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High-grade non-Hodgkin lymphoma

High-grade non-Hodgkin lymphoma

About our information


The Lymphoma Association is committed to the provision of high-quality information for people with lymphoma, their families and friends. We produce our information using nationally recognised guidelines, including the DISCERN tool for information about treatments, the NHS Toolkit for producing patient information, and the Campaign for Plain English guidelines. The Lymphoma Association is an accredited member of The Information Standard independent quality assurance scheme, which is supported by the Department of Health. Our publications are written by experienced medical writers, in close collaboration with medical advisers with expertise in the appropriate field. References are provided where academic texts have been used. Some publications are written by professionals themselves, acting on guidance provided by the Lymphoma Association. In some instances, our publications are funded by grants from pharmaceutical companies. These sponsors do not have any involvement in the content of a publication and have no editorial input. This booklet has been sponsored by Roche Products Limited.

The information in this booklet can be made available in large print.

Introduction
You may be someone with high-grade non-Hodgkin lymphoma. Perhaps someone close to you has lymphoma. You are not alone: non-Hodgkin lymphoma is the fifth most common cancer in the UK more than 12,000 people are diagnosed with it each year. High-grade non-Hodgkin lymphoma is a term that refers to a range of different fast-growing lymphomas. These lymphomas require immediate treatment but most are potentially curable. People often want to know more about their own particular kind of high-grade lymphoma. This booklet has a section describing many of the types of high-grade lymphoma and highlighting how they differ from other lymphomas. Because we have covered many different treatments and many different types of high-grade lymphoma, we dont recommend that you read the whole of this booklet from cover to cover. In Parts 3 and 4, we suggest that you read only the sections that refer specifically to your particular type of high-grade non-Hodgkin lymphoma or to treatments that you are going to have. Looking at sections about other lymphomas could be confusing or even misleading. This booklet is about high-grade non-Hodgkin lymphoma in adulthood. We produce other information about lymphomas in children and a booklet specifically for young people with lymphoma. Please ring our helpline (0808 808 5555).
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Acknowledgements
This booklet is a revision of a booklet first published in 2006. We would like to acknowledge the continued support of our Medical Advisory Panel, Lymphoma Nurse Specialists and other expert advisers, whose ongoing contributions help us in the development of our publications. In particular we would like to thank the following experts for their assistance in the 2012 revision of this booklet: Dr Chris Hatton, Consultant Haematologist and Clinical Director of Haematology, Oxford Radcliffe Hospitals NHS Trust Frances Penny, Lymphoma Clinical Nurse Specialist, Royal Free London NHS Foundation Trust Finally, thank you to all those people affected by lymphoma who have helped us by making suggestions on what to include or by reviewing the revised text.

Disclaimer
We make every effort to ensure that the information we provide is accurate but it should not be relied upon to reflect the current state of medical research, which is constantly changing. If you are concerned about your health, you should consult your doctor. The Lymphoma Association cannot accept liability for any loss or damage resulting from any inaccuracy in this information or third party information such as information on websites which we link to.
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Contents
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Understanding high-grade non-Hodgkin lymphoma
7 The lymphatic system 8 What is lymphoma? 9 How and why do lymphomas develop? 10 What are the symptoms of lymphoma? 11 How are lymphomas classified? 12 What does T-cell or B-cell non-Hodgkin lymphoma mean? 13 What does high-grade lymphoma mean? 13 Summary 14

Diagnosis and staging

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How is lymphoma diagnosed? 16 Having a biopsy 16 What other tests may be required? 17 Blood tests 18 Bone marrow biopsy 18 Imaging 19 Lumbar puncture 22 What does the stage mean? 23 Summary 24

Treatment of high-grade non-Hodgkin lymphoma

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About your treatment 26 Planning treatment 26 Where will my lymphoma be treated? 27 Research and clinical trials 28 Chemotherapy 30 What is chemotherapy? 30 How chemotherapy is given 30 What are the side effects of chemotherapy? 35 Other treatments sometimes given with chemotherapy 43 CHOP chemotherapy 44 Other chemotherapy regimens 45 Antibody therapy 46 What is antibody therapy? 46 How is antibody therapy given? 47 Side effects of antibody therapy 47 Radiotherapy 48 What is radiotherapy? 48 How is radiotherapy given? 48 What are the side effects of radiotherapy? 50 Stem cell transplants 53 What happens after treatment? 55 Remission and cure 55 Follow-up appointments 55 What happens if my lymphoma comes back? 56 Summary 57

Types of high-grade non-Hodgkin lymphoma

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B-cell non-Hodgkin lymphomas 61 Diffuse large B-cell lymphoma 61 Burkitt lymphoma 64 Mantle cell lymphoma 67 Primary mediastinal large B-cell lymphoma 71 Primary central nervous system lymphoma 72 T-cell non-Hodgkin lymphomas 75 Lymphoblastic lymphoma 75 Peripheral T-cell lymphoma 78 Anaplastic large cell lymphoma 80 Angioimmunoblastic T-cell lymphoma (AITL) 82 Enteropathy-associated T-cell lymphoma 84 Adult T-cell leukaemia/lymphoma 86 Nasal-type NK/T-cell lymphoma 88 Immunodeficiency-associated non-Hodgkin lymphomas 90 Post-transplant lymphoproliferative disorder 90 HIV- and AIDS-related lymphoma 92 Conclusion Glossary Useful organisations Selected references Appendix: Tips for coping with side effects 94 95 100 104 106

Understanding high-grade non-Hodgkin lymphoma


The lymphatic system What is lymphoma? How and why do lymphomas develop? What are the symptoms of lymphoma? How are lymphomas classified?

High-grade non-Hodgkin lymphoma

The lymphatic system

Neck (cervical) lymph nodes Lymph vessels Armpit (axillary) lymph nodes Thymus Diaphragm (muscle that separates the chest from the abdomen) Spleen Liver

Groin (inguinal) lymph nodes

Understanding high-grade non-Hodgkin lymphoma

Our lymphatic systems are made up of a complex network of tubes (known as lymph vessels), glands (known as lymph nodes) and other organs such as the spleen. We have lymph nodes and lymph vessels throughout our bodies. Some groups of lymph nodes may be easily felt, particularly under the arms, in the neck and in the groin; others can only be seen on scans. The lymphatic system is part of the bodys natural defence against infection the immune system. The lymph nodes are an important part of this, acting as a sieve in the lymphatic system. They are a home to large numbers of lymphocytes (a type of white blood cell that helps our bodies to fight infection).

What is lymphoma?
Lymphomas are cancers of the lymphatic system. They occur when some of the lymphocytes become cancerous. Lymphoma is not just one illness. It describes a wide range of diseases that all start with a cancerous lymphocyte. The condition was first identified in the 19th century by Dr Thomas Hodgkin. One type of lymphoma known as Hodgkin lymphoma (or Hodgkins disease) is named after him; other types are known as non-Hodgkin lymphoma. Each year almost 12,000 people are diagnosed with non-Hodgkin lymphoma in the UK. It can occur in people of any age, including children, but it becomes steadily more common in people aged 50 and over.
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High-grade non-Hodgkin lymphoma

How and why do lymphomas develop?


Lymphocytes are constantly dividing to make new lymphocytes. When fighting an infection, young and immature lymphocytes divide faster to make many more cells. Only those that are very specific for the target infection are useful to the immune system. Any lymphocytes that do not target the particular infection will usually die, which means normally only useful lymphocytes survive. Lymphomas can result when there is a breakdown of these normally carefully controlled processes. Instead of dying in the normal way, untargeted rogue lymphocytes divide in an uncontrolled fashion. This leads to the development of a lump, most often in a lymph node a lymphoma. The rogue lymphocytes may also collect in other places, such as the spleen, liver, gut, skin and bone marrow. Such sites (known as extranodal sites) can also be involved in some cases of lymphoma. In most cases, the exact trigger that causes the changes and makes the lymphocytes become cancerous remains unknown. Despite this, it is important that you know: You have not done anything to yourself to cause lymphoma. You did not inherit it from your parents. You didnt catch it and you cant pass it on to others.

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Understanding high-grade non-Hodgkin lymphoma

Although anyone can develop a lymphoma, some people are more at risk of lymphoma. This may be because of another medical condition, particularly conditions that affect the immune system. For example, people who are on long-term drugs after an organ transplant, people who take immunosuppressants for other disorders and people living with HIV (human immunodeficiency virus) infection and AIDS (acquired immune deficiency syndrome) are more at risk of developing certain types of lymphoma. Some kinds of lymphoma are known to be related to particular viruses. Research studies have investigated other possible causes of lymphoma, such as chemicals present in the working environment or the increased risk of lymphoma in some families. But it will be some time before scientists fully understand why lymphomas develop.

What are the symptoms of lymphoma?


People with lymphoma can have many different symptoms. Some people have no symptoms at all. But more symptoms will occur and these will get worse if the illness is not treated. Some of the symptoms are common to many types of cancer. For instance, the lymphoma cells take up energy and nutrients that are needed by healthy cells so people often feel very tired. The commonest symptom of lymphoma is: a painless lump or swelling, often in the neck, armpit or groin a swollen lymph node.
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Other possible symptoms include: unexplained weight loss excessive sweating at night persistent fevers and flu-like symptoms skin rash or itching

these are known as B symptoms

Lymphomas can affect any organ of the body such as stomach, bowel, skin and brain, among others. The symptoms may be related to the part of the body that is affected, for example: abdominal pain diarrhoea or change in bowel habit jaundice a persistent cough or breathlessness if the lung is affected.

How are lymphomas classified?


Lymphomas are classified (or divided and sorted) into different types. They are divided in three broad ways: Hodgkin lymphoma or non-Hodgkin lymphoma T-cell or B-cell non-Hodgkin lymphoma high-grade or low-grade non-Hodgkin lymphoma. Doctors will classify your lymphoma by checking: what the cells look like under the microscope what chemicals or proteins are on the surface of the cells what changes have happened to the genes in the cells. The classification of your lymphoma is very important. It will give your specialist vital information about your
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Understanding high-grade non-Hodgkin lymphoma

illness and what you can expect. In particular it will tell your specialist: what type of lymphocyte has become cancerous whether the lymphoma is growing quickly or slowly how the lymphoma may behave, for example what parts of your body are likely to be affected what kind of treatment will be needed.

What does T-cell or B-cell non-Hodgkin lymphoma mean?


As previously discussed, lymphomas happen when a lymphocyte divides in an uncontrolled manner, meaning it has become cancerous. Lymphocytes may be either B cells or T cells and both types may become cancerous. B cells and T cells mature in different parts of the body: B cells in the bone marrow; T cells in the thymus (a gland found in your chest). Both types help protect our bodies from infection and illness but do so in slightly different ways. High-grade non-Hodgkin lymphomas will be either B-cell lymphomas or T-cell lymphomas. B-cell lymphomas are about 10 times more common than T-cell lymphomas.

What does high-grade lymphoma mean?


Lymphomas are described as high grade if the cells appear to be dividing quickly. This means the lymphoma is a fast-growing type. You may also hear the word aggressive used to describe high-grade lymphoma. This may sound alarming but actually many high-grade lymphomas have a better chance of being cured than other low-grade or slow-growing lymphomas.
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Summary
Lymphomas are cancers of the lymphatic system. There are many different types of lymphoma. Lymphomas develop when some of the lymphocytes become cancerous and start to grow out of control. The causes of lymphoma are not fully understood. Lymphomas may cause very few symptoms. The commonest symptom is a painless lump or swollen lymph node. Other symptoms include weight loss, night sweats and tiredness. Lymphomas are either Hodgkin lymphoma (Hodgkins disease) or non-Hodgkin lymphoma. Non-Hodgkin lymphomas can be fast growing (high grade) or slow growing (low grade). They may develop in B cells or T cells. B-cell lymphomas are more common than T-cell lymphomas. If you would like to talk to us about lymphoma please ring our helpline (0808 808 5555).

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Diagnosis and staging


How is lymphoma diagnosed? What other tests may be required? What does the stage mean?

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How is lymphoma diagnosed?

The word diagnosis simply means finding out what is wrong. In most cases, it is not possible for a GP to confirm whether or not you have lymphoma not even from a blood test. Your GP will send you to a doctor at a hospital for further tests. These will almost certainly include a biopsy.

Having a biopsy
In most cases a biopsy is the only way to tell whether or not a lump is lymphoma. It is a test that removes some of your cells so they can be looked at under a microscope. The method used to obtain your biopsy will depend on where your nodes are found and the preferred practice in your hospital. If you have an enlarged lymph node that can be easily felt, it may either be removed completely or partly by a surgeon (an excision biopsy) or a small sample of the lymph node (a core biopsy) may be taken. If you have an excision biopsy, you are likely to need a general anaesthetic and a short stay in hospital. A core biopsy is usually taken with ultrasound guidance by a radiologist (a specialist in X-rays and scans). It usually needs a local anaesthetic and you will probably be able to go home the same day. If your only nodes are internal, either part or all of a node will be removed laparoscopically (key-hole surgery) or a core biopsy will be taken by a radiologist. For laparoscopic surgery, you will need to have a general
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Diagnosis and staging

anaesthetic and may need to stay in hospital. If a core biopsy is done, it will be taken whilst scanning to make sure the abnormal area is biopsied. Again you will probably only need a local anaesthetic and may be able to go home the same day. Sometimes the biopsy will be taken from other areas that may be affected, such as the bone marrow. The biopsy is examined by a specialist pathologist experienced at diagnosing lymphomas. Further tests may be required to define more accurately the exact type of lymphoma that you have. These tests give more information about the cells, for instance about changes to their DNA or to the proteins on their surface.

What other tests may be required?


Your doctors may want you to have other tests as well as your biopsy. This section describes some of the common tests for lymphoma, but dont be worried if you dont have all of them. Sometimes only a few of the tests are needed to find out about your lymphoma. Most of these tests will be done as an outpatient, meaning you wont need to stay in hospital overnight. It may take a couple of weeks for all the results to be available. It is normal for you to feel anxious whilst waiting for these tests and the results. Its very important though that your doctors have all the information they need about your lymphoma. This will help them choose the most suitable treatment for you.
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Blood tests

Blood samples will be taken before you start treatment and at regular intervals during treatment. Blood tests may be done for the following reasons: to check for anaemia or other low blood counts to check that your kidneys and liver are working well to provide information on how your lymphoma may behave to look for infections such as hepatitis or HIV, which may also need treatment or could flare up with lymphoma treatments.

Bone marrow biopsy


The bone marrow is spongy, jelly-like material found in the middle of the large bones in our bodies. The bone marrow is responsible for making our blood cells. A bone marrow biopsy may be needed to look for lymphoma cells, which are sometimes found in the bone marrow in people with high-grade lymphoma. You may hear this test referred to as a bone marrow aspirate or bone marrow trephine. The test is quite simple, but if you are taking medicines to thin your blood youll probably be asked to stop these beforehand. A sample of bone marrow is taken usually from the bone at the back of your pelvis (above your hip). The area is first numbed with a local anaesthetic. The biopsy needle is then inserted through the skin and into the bone. Despite the local anaesthetic, taking the sample can still be painful. You may need to take painkillers before and after your bone marrow biopsy. If youre very anxious,
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Diagnosis and staging

it may be possible for you to be given a sedative. But youll need to discuss this with your doctor as it isnt advisable for some people. The whole procedure takes around 15 20 minutes and not long afterwards youll be able to go home. A small dressing will cover the biopsy area at first and youll be given advice about when this should be removed.

Imaging
X-rays X-rays can be used to look at various parts of your body. For example, a chest X-ray may be used to see if there are any enlarged lymph nodes in your chest. X-rays are painless and shouldnt take longer than a few minutes. CT scans Computed tomography (CT) scans use a series of X-rays to create pictures of your body in cross-section, like slices through your body. The test involves lying on a padded table that moves your body into a doughnut-shaped camera. As the table moves, the camera takes pictures of the different layers of your body. The scan is painless and usually takes a few minutes. You will be asked to lie quite still while the pictures are being taken. You might also be asked to hold your breath for up to 20 seconds at some stage during the scan. You may be asked not to eat or drink on the morning of the scan. This is because most people will be given a special liquid dye (a contrast agent) either to drink or as
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an injection into a vein beforehand. These make it easier to see some of your internal organs.

The drink or injection might make you feel hot all over but this usually only lasts a few minutes. Tell someone if you feel this way. You should also tell the staff, before you have the drink or injection, if: you are allergic to iodine you have had a previous reaction to a contrast agent you have asthma, emphysema or severe heart disease. MRI scans Magnetic resonance imaging (MRI) scans are similar to CT scans, except they create pictures using strong magnets instead of X-rays. The pictures are slightly different and are particularly good for looking at certain tissues such as the brain. Youll be asked to lie on a padded table that moves you into a cylinder (tube). The cylinder measures magnetic waves as they pass through your body. The test is painless but can take up to an hour. The scanner can be very noisy and as you are in a small space, you may feel claustrophobic. Because the cylinder uses a magnet, youll be asked to take off all metal jewellery and any items of clothing that possibly have metal parts, such as some bras, beforehand. The staff will also ask you about any other metal implants (such as a hip replacement or a pacemaker) or recent operations. Occasionally, metal staples are used instead of stitches after an operation, so mention this if you still have them in after your biopsy.

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Diagnosis and staging

PET scans Positron-emission tomography (PET) scans give more detailed information about cell activity. They are particularly good for helping doctors work out which cells are cancerous and which are not. They use a radioactive form of sugar to look at how active the cells are. More active cells, like cancer cells, take up more sugar than normal cells. The radioactivity within the cancer cells is then detected with a special camera. Nowadays, PET/CT scans are usually done. These combine a PET scan with a CT scan to give a clearer picture of exactly which areas are cancerous. Having the scan is painless and very like having a CT scan. The whole process usually takes about 2 3 hours. You will be asked not to eat or exercise before your scan. When you arrive for the scan, the special sugar will be injected into a vein. Youll then be asked to rest for an hour or more while the cells take up the sugar. Note: if you are diabetic, you should tell the scanning centre staff that you have diabetes before you attend. You should be given specific instructions about managing your diabetes on the day of the scan. PET/CT scans are increasingly being used before treatment (to help decide which treatment is best) or after treatment (to see what response there has been). They are now available in most NHS cancer networks. Some hospitals are visited by mobile PET/CT scanners on a regular basis. In other areas, you may have to travel to your nearest hospital with a scanner.

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Lumbar puncture

Your brain and spinal cord are cushioned by a fluid called the cerebrospinal fluid (CSF). Lymphoma cells can be found in the CSF in some kinds of high-grade non-Hodgkin lymphoma. Your doctor may want to look at your CSF with a microscope to see if any lymphoma cells are present. A test called a lumbar puncture (spinal tap) will be done to take a small sample of your CSF . You can eat and drink as normal before the test. But if you are taking medicines to thin your blood, you may be asked to stop these beforehand. Typically, while the test is being done, you will be asked to lie on your side with your knees pulled up towards your chest. A doctor will feel for a gap between the bones of the lumbar part of your spine (in the small of your back). You will be given an injection of local anaesthetic. A special needle will then be passed through the gap into the space containing the CSF . A small amount of your CSF (about 3 4 teaspoons) will be drained off and sent to the laboratory for testing. Apart from the initial sting of the local anaesthetic, you shouldnt find this test painful. You will though be asked to keep very still during the test. Afterwards, youll need to stay lying down for a period of time (sometimes up to 4 hours). This will reduce the chances of you developing a headache, which is common after a lumbar puncture.

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Diagnosis and staging

What does the stage mean?


Once all the test results are ready, your doctor will be able to tell which parts of your body are affected by your lymphoma. This is called the stage of your disease. It will be an important factor in planning your treatment. Stage I Stage II Stage III One group of lymph nodes affected Two or more groups of lymph nodes affected on one side of the diaphragm* Lymph nodes affected on both sides of the diaphragm*

Stage IV Lymphoma can be found either in organs not in the lymphatic system or in the bone marrow
*a sheet of muscle that separates the chest from the abdomen

In addition to the stage, you may also hear the letters A or B used to describe your illness. B would mean you have B symptoms (marked weight loss, drenching night sweats or unexplained fevers); A would mean you have none of these. In some people, the lymphoma is found only in places outside the lymphatic system. Your doctors may add the letter E for extranodal to refer to lymphoma that is outside the lymphatic system. An important extranodal area that can be affected by some lymphomas is the central nervous system (CNS). This is made up of the brain and spinal cord. The presence of disease in your CNS or the chances of
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you developing it later may be taken into account when planning your treatment.

Stage I lymphoma, and sometimes stage II, may be referred to as early-stage disease. Stages III and IV lymphoma, and sometimes stage II, are referred to as advanced-stage disease. This may sound alarming but there are good treatments available for lymphoma at all disease stages. High-grade lymphoma is not like many other cancers where advanced stage may mean the disease is incurable and treatment options are limited.

Summary
Lymphoma is normally diagnosed by a biopsy. This means that cells are removed to be looked at under the microscope. Usually an enlarged lymph node is sampled either by removing the whole or part of it or by taking a core biopsy. Specialised tests are performed on the sample to identify what type of lymphoma you have. You may need to have other tests, which may include blood tests, a bone marrow and imaging tests. The results allow your doctors to work out the stage of your lymphoma. This information is important in planning the right treatment for you.

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Treatment of high-grade non-Hodgkin lymphoma


About your treatment Chemotherapy Antibody therapy Radiotherapy Stem cell transplants What happens after treatment?

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About your treatment


Planning treatment
Once your doctors have the results of the tests, they will have the information needed to plan your treatment. The best treatment for you will depend largely on: the stage of your disease the classification of your disease. Other important factors that your doctors will take into account include: your age your general health and the health of your major organs your blood test results. Sometimes these factors (known as prognostic indicators) are put together to give a prognostic score. All this information helps the doctors to plan the treatment that is best for your individual situation. Although you will have one lead consultant responsible for your care, the plan for your treatment will be discussed in a multidisciplinary team (MDT) meeting. At this meeting a number of specialists will discuss the most appropriate treatment for you on the basis of your test results. Completing the tests and planning your treatment can take a couple of weeks. This might seem a long time, but the information being collected is very important. Your doctor needs to know as much as possible about your illness before choosing the treatment that will be best for you. It is natural to feel frustrated or worried when you are waiting to find out more. You may find it helps to talk about this to your specialist nurse or your GP .
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Where will my lymphoma be treated?


People with lymphoma may be treated at local hospitals or at larger hospitals with cancer centres. Sometimes people will have their treatment shared between the two. Your GP , or the doctor who finds out that you have lymphoma, will send you to the nearest hospital with a lymphoma specialist. If you have a rare type of lymphoma you may be referred to a specialist hospital. Larger specialist treatment centres may have more experience of treating rarer lymphomas because they are likely to have seen more cases. Your local specialist might also treat you nearer home after agreement at the MDT meeting and with guidance from the larger centre. A lymphoma specialist can be a haematologist (someone who specialises in diseases of blood cells) or an oncologist (someone who specialises in cancers). Lymphoma experts come from both these fields of medicine. Your doctor will not mind if you want to ask questions about your hospital. Some of the questions you might like to ask are: Will your doctor meet regularly with other lymphoma specialists at an MDT meeting? Does the hospital have a clinical nurse specialist or other specialist cancer nurse? Does the hospital have a laboratory on site for fast blood test results? Does the hospital participate in clinical trials?
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Does the hospital have close links with specialist


lymphoma pathologists? What other experts are there to help if you need them? For example, will you be able to see a counsellor if you need to?

Many people find it helps to take a relative or friend along with them to their hospital appointments. Your companion may remember parts of the discussion that you dont or may think of other questions or concerns that they know you have. Your doctor wont mind, in fact most will encourage their patients to bring someone along. For more information about where to be treated or seeking a second opinion please ring our helpline (0808 808 5555).

Research and clinical trials


You may be asked if you would like to take part in a clinical trial. These are scientific studies involving human subjects. Some trials are designed to test new treatments that havent yet been tried in lymphoma. Many of the trials with the largest number of people are trying to work out the best ways to use existing treatments. Clinical trials are very important in improving the future treatment of people with lymphoma. Clinical trials are usually specific to a type of lymphoma. There will also be other criteria for inclusion into clinical trials, so they are not suitable for everyone.
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Not all hospitals conduct clinical trials. This may be something to discuss with your doctor when planning treatment. Clinical trials are entirely voluntary. You do not have to take part in a trial if you do not wish to. You can always opt to have the standard treatment if you prefer. It is important you understand fully what is involved before you agree to take part in a trial. You should be given written information. Should you later change your mind, you are free to withdraw at any time and go back onto standard treatment. You may not get any benefit yourself from taking part in a trial no one can say which treatment is better until the trial is completed. You may be more closely monitored or have more tests because of being in the trial. The one certain fact is that you will be helping other people get the best possible treatment in the future. We have an information booklet about clinical trials. Please call our helpline (0808 808 5555) if you would like a copy or if you would like to talk to us about your treatment.

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Chemotherapy
What is chemotherapy?
The word chemotherapy literally means treatment with drugs. Drugs for cancer are called cytotoxic because they kill cells: cyto means cell, and toxic means poisonous. Chemotherapy works by stopping the cancer cells dividing. Different chemotherapy drugs target cells in different ways. Chemotherapy for lymphoma usually involves giving more than one kind of drug. This increases the chances of killing as many cancer cells as possible. Chemotherapy works particularly well on cells that are dividing quickly and less well on cells that are dormant (resting). To target as many cells as possible, chemotherapy is generally given as repeated courses (often called cycles). With each treatment cycle more cells are destroyed and the lymphoma gradually shrinks. Each cycle also has a rest period when no chemotherapy is given. This is because normal cells, such as those in the bone marrow and the lining of the mouth and bowel, are also damaged by chemotherapy. The rest period allows the healthy normal cells time to recover.

How chemotherapy is given


Chemotherapy for high-grade non-Hodgkin lymphoma is given as a number of cycles, each of which takes a few weeks. A number of cycles (often 68) will be given over a period of several months. The exact timetable for your treatment will depend on what kind of chemotherapy you are having. Your hospital team will give you specific information about your treatment and what to expect.
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Chemotherapy for high-grade lymphoma is often given as an outpatient treatment. This means you will visit hospital on the day of treatment but go home afterwards. Some types of chemotherapy can only be given to inpatients. This means you will need to stay in hospital. Your specialist will discuss this with you when explaining the treatment they are recommending for you. Chemotherapy drugs for high-grade lymphoma can be given in a number of different ways: intravenously, meaning through a tube into a vein (the most common way) orally, meaning by mouth in tablet or capsule form intrathecally, meaning into the fluid that surrounds your brain and spinal cord (less often needed). Intravenous chemotherapy Intravenous drugs are typically delivered through a cannula (a small tube placed in one of your veins). The nurse or doctor will put the cannula into a vein, usually on the back of your hand or in your lower arm. The cannula is made up of a soft plastic tube and a needle within it. The needle goes into the vein first and then is taken out, leaving only the soft tube in the vein. This is secured with an adhesive dressing to keep it in position. Usually, the cannula will be taken out before you go home and another one put in next time. Some intravenous drugs are given as a bolus or a push dose. This means that the nurse injects the drug through the cannula over a short period of time.

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Other intravenous drugs are given as an infusion (a drip). These are mixed with another fluid in a bag, and the bag is set up to drip through the cannula over a specified period of time. The bag needs to be kept higher than your arm, so will usually be hung on a metal pole. Often the pole is on wheels, so you can walk around and visit the toilet if you need to while the drip is connected. You might have more than one bag going at the same time. You might need to go to the toilet more often as you may be given a lot of liquid over a relatively short time. Drugs given as infusions are often controlled with pumps (small machines to maintain the correct speed of flow) that can be attached to the pole. They may beep from time to time to let the nurses know if something is not right. This might sound alarming at the time, but dont worry as the drip will stop until the problem is sorted out. Intravenous chemotherapy can sometimes make your arm sting. Do tell the nurse if you feel any discomfort. Some people will need to have their intravenous chemotherapy given through a central venous catheter (a more permanent intravenous tube) instead of a small cannula. These make it easier to give drugs and other fluids over a longer period of time and to take blood samples without the discomfort of repeated needles. Mostly they are used for people having certain types of chemotherapy (usually those spread over longer periods of time). They may also be used for people who have particular difficulties with normal cannulas being put in.

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There are two types of central venous catheter: a PICC line (peripherally inserted central catheter) or a tunnelled central line. Both types end in a vein that is further inside your body. A PICC line usually goes in through a vein in your arm at the level of your elbow. Its held in place by a tape or dressing. A tunnelled central line is usually positioned on your upper chest. Part of the line runs in a tunnel under your skin, which reduces the risk of infection. You may hear these called a Hickman or Groshong line. Central venous catheters are designed to stay in for longer, hopefully for all of your treatment. The line will be covered to protect it when you go home and you will be given instructions about how to care for it. Despite this, lines can become infected. If you develop a temperature, feel shivery or if the area around the line becomes red or sore, you should contact your hospital immediately. Oral chemotherapy You may be taking chemotherapy that can all be given orally. In this case, your treatment will be prescribed by the doctors in the outpatient or day treatment clinic and you will most likely collect it from the hospital pharmacy to take home with you. Some people will also be asked to take oral chemotherapy as part of a combination regimen. The person giving you the medicines should tell you what to take and when. This information will also be on the boxes or bottles containing your treatment. You may also be given special instructions or advice about how you should store the tablets or capsules and/or how you

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should take them. It is important that chemotherapy tablets are not handled by anyone other the person who is taking them. If you are a carer assisting someone to take their chemotherapy, you should wear gloves when handling any tablets.

Intrathecal chemotherapy Some kinds of high-grade non-Hodgkin lymphoma may involve the CNS (the brain and spinal cord). The CNS is surrounded by a fluid that cushions it called cerebrospinal fluid (CSF). The CNS is protected by something called the blood brain barrier. This stops many drugs crossing from the bloodstream into the CSF . One way to treat your CNS is to give chemotherapy directly into the CSF . This is known as intrathecal chemotherapy. If your doctors think you are at risk of your lymphoma spreading to your CNS, they may recommend you have intrathecal chemotherapy as a preventive measure. This is known as CNS prophylaxis. For more information about either lymphomas in the CNS or CNS prophylaxis please ring our helpline (0808 808 5555). Intrathecal chemotherapy is usually given during a lumbar puncture. It is given by a senior doctor who is specially qualified to give intrathecal chemotherapy. You will find lumbar punctures described in more detail on page 22.

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Treatment of high-grade non-Hodgkin lymphoma

What are the side effects of chemotherapy?


It is not possible to say what side effects you will have from your treatment. Everybody will get slightly different side effects, even if they are having the same treatment as someone else. The side effects of chemotherapy will depend on what drugs you are having. You will be given information about what to expect. If you would like to talk about the side effects of chemotherapy please ring our helpline (0808 808 5555). Most treatment side effects are short term. Some may be long term or permanent. Your doctors should discuss this with you before treatment starts. It is important to let your hospital team know about your side effects. Tell them if any of the side effects change during your treatment. Your doctors and nurses wont always ask how you are feeling, and they might assume you are OK if you dont say anything. There are usually things that can be done to help with side effects. The following are some of the more common side effects of treatment. It is not possible to discuss every side effect in this booklet. Do discuss with your hospital team if you have concerns about side effects not mentioned here. You will find some tips for coping with these side effects in the appendix at the end of this booklet.
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Risk of infection and low blood counts The most important side effect of chemotherapy is damage to the bone marrow. The bone marrow is responsible for producing the bodys blood cells. These include your white blood cells that help to fight infection. The most important white blood cell is called a neutrophil. You may hear a low white cell count referred to as neutropenia, meaning the number of neutrophils is low. This is a common occurrence after many types of chemotherapy for high-grade non-Hodgkin lymphoma. It means you will be more at risk of infection. The white cell count tends to be lowest about 710 days after each dose of chemotherapy, but it can last for some time. If you are neutropenic (have neutropenia), you are at more risk of infection. If you develop an infection, it can be serious, sometimes even life-threatening. You should contact your hospital immediately if you develop signs of an infection. You will find the possible signs described in the appendix on page 106. If your white cell count is too low, it may be necessary to delay your next cycle of treatment until your blood count has had a chance to recover. You may need to have other drugs to help boost your blood count. These are called growth factors or G-CSF. Growth factors are described in more detail on pages 43 44.

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Treatment of high-grade non-Hodgkin lymphoma

Sometimes other blood cells can be affected by chemotherapy. Your red blood cells carry oxygen. A shortage of these is called anaemia, which can make you feel tired or short of breath. Platelets help to stop you bleeding. A shortage of these is called thrombocytopenia. You will have regular blood tests taken to check your blood counts during your treatment. Change in taste and other dietary problems It is quite common for people being treated with chemotherapy to have some type of problem with eating or diet. These may include: finding food starts to taste different losing your appetite nausea a sore mouth that makes it difficult to eat. Feeling sick Some chemotherapy drugs may make you feel sick (or nauseous) after treatment. You might vomit too. You will be given anti-sickness drugs to help. These are called antiemetics. Tell your specialist or nurse if you are feeling sick. If you are taking anti-sickness drugs that arent working, tell one of your hospital team. Sometimes a change in the anti-sickness drug is needed. Fatigue Fatigue is one of the most common experiences for people with lymphoma. Cancer-related fatigue is quite different from normal tiredness. It can mean that you find it difficult to concentrate or make decisions. It may make you short-tempered. You might sometimes

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High-grade non-Hodgkin lymphoma

feel too tired to do even simple things, like watching television. Fatigue can be difficult to describe and you may find that other people (even your doctors) dont really understand it.

Fatigue is thought to be the result of a combination of factors, which may include: side effects of your treatment the effect of the lymphoma on your body (using up your energy) a low red cell count (anaemia) not eating or sleeping well feeling anxious or depressed. It may be some months after treatment before your fatigue goes away. For more information about fatigue please ring our helpline (0808 808 5555). Hair loss Some chemotherapy drugs used to treat high-grade lymphoma commonly cause hair loss. This means it is likely you will lose some or all of your hair. Hair loss will typically begin within a couple of weeks of starting treatment. It can fall out quite suddenly, which can be very distressing. Ask your team about wig facilities that are available on the NHS in your area. Your hair will usually start to grow back within a month or two of your treatment finishing. Sometimes it may seem different, perhaps curlier or a slightly different colour. It will probably take 6 12 months for it to return to its previous thickness.

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Treatment of high-grade non-Hodgkin lymphoma

Sore mouth Chemotherapy drugs may give you a sore mouth. This is because the chemotherapy damages the cells of the lining of your mouth. This is called mucositis and can be painful. It can also make you more prone to mouth ulcers and infections. Your hospital team will be able to advise you about mouthwashes that can help. Peripheral neuropathy Some chemotherapy drugs cause damage to the nerves that carry information about touch, temperature, pain and sensation. The drugs can also damage the nerves involved in muscle movement. This is called peripheral neuropathy. Peripheral neuropathy is usually related to particular groups of drugs. One drug that is often used in high-grade lymphoma and may cause neuropathy is vincristine. Peripheral neuropathy commonly affects the nerves in your hands and feet, but you might feel it in other places too. It can cause: pins and needles pain numbness clumsiness problems with balance increased sensitivity to heat constipation. Symptoms of peripheral neuropathy usually develop soon after you start treatment, but not everyone will get them. If you do have any of these symptoms, you

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should tell your doctors or nurses before you have your next dose of chemotherapy. They might want to make a change to your treatment to prevent these problems getting worse.

For most people symptoms of peripheral neuropathy will be temporary, but some people will experience long-term or permanent damage. Bladder problems The drugs cyclophosphamide (particularly in a high dose) and ifosfamide (Mitoxana) can cause irritation and bleeding from the lining of the bladder and the kidneys. You will need to drink lots of extra fluid with these treatments. You may also have extra fluid through your drip. A drug called mesna (Uromitexan) can be given to prevent bladder and kidney complications. It is given with ifosfamide and with cyclophosphamide when used at a high dose. If you notice blood in your urine, you should tell one of your hospital team straight away. Reduced fertility Some treatments for lymphoma may reduce your fertility. Many people are able to have children after treatment without any problem, but certain chemotherapy drugs, radiotherapy to an area that includes the pelvis in women and testes in men or high-dose chemotherapy used in stem cell transplants can make this less likely.

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Treatment of high-grade non-Hodgkin lymphoma

It will not be possible to say for certain how your fertility might be affected, but your consultant should discuss this with you before your treatment starts. Men may wish to consider sperm storage before they start treatment. Even though serious illness can reduce your sperm count, developments in fertility treatment make this worthwhile for many men who may wish to have children in the future. The options for women are more limited. This is partly because fertility treatments in women take more time and could mean a potentially risky delay in starting lymphoma treatment. Embryo storage may be an option for some women who are fit enough to undergo the hormonal treatments needed before egg collection. Egg storage and ovarian tissue freezing may be offered at some hospitals. These are still experimental techniques and, at present, are much less successful than sperm storage. You will need to discuss what options are available to you with your consultant. Different healthcare authorities will have different policies about the funding of such treatments on the NHS. Reduced fertility in women is related to age. Women closer to the age of normal menopause are more likely to experience reduced fertility and may also undergo an early menopause. You may find that your periods become irregular or stop altogether during treatment. Afterwards they may return to normal, but some women will find that the irregularity continues.
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High-grade non-Hodgkin lymphoma

Although chemotherapy may make you less fertile, doctors generally advise that women with lymphoma do not become pregnant during their treatment and for some time afterwards (many doctors recommend 2 years) and that men with lymphoma avoid making their partner pregnant whilst they are receiving chemotherapy and for at least 3 months afterwards. This is because treatments may damage sperm or eggs and could be harmful to a developing baby. Also if a woman is pregnant it can make it more difficult to treat the lymphoma. However, everybodys circumstances will be different, so it is important to discuss such issues with your hospital team. This will allow them to give specific advice on what they feel would be best for you. There is no reason why you should not have sex during treatment if you feel like it, but you should use contraceptives during treatment. Oral contraceptive tablets may be less effective whilst you are on treatment, so do discuss this with your doctor or nurse. Traces of chemotherapy may remain in the body possibly for up to 5 days following treatment. Condoms should be used during this time and you should avoid oral sex as body fluids may contain traces of chemotherapy. For more information about sex, fertility or early menopause please ring our helpline (0808 808 5555).

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Treatment of high-grade non-Hodgkin lymphoma

Other treatments sometimes given with chemotherapy


Steroids The word steroid refers to a large family of similar drugs that imitate hormones produced naturally by our bodies. The steroids used in lymphoma treatment help to kill the lymphoma cells. They also reduce nausea and help you to feel better. Steroids are usually given as tablets, often in the form of prednisolone. Steroids can have many different side effects and extra care may be needed using them in people who have diabetes. The side effects may depend on the dose used and whether the steroids are given for a short or long time. For more information about steroids please ring our helpline (0808 808 5555). Growth factors Growth factors are sometimes used in the treatment of high-grade non-Hodgkin lymphomas. They do not treat the cancer cells, but help to maintain your white blood cell count when you are having chemotherapy. Growth factors are hormones that occur naturally in our bodies. The growth factor most commonly used is called G-CSF. It stimulates the production of stem cells in your bone marrow and helps the marrow to produce more white blood cells. G-CSF is given as an injection into the fatty tissue under your skin (known as a subcutaneous injection). Most people learn to give the injections themselves or a relative may be taught how to give them. The injections use a tiny needle. They are given into your stomach, the top of your leg or the top of your arm.
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G-CSF can cause side effects such as flu-like symptoms, back pain and headaches. You should ask your hospital team about what you can take to help with this. The injections can sting. Some brands are stored differently, but if you have one that must be kept in the fridge it may help if you take the injection out of the fridge 20 minutes before you use it. If you have any unusual symptoms or feel unwell during your growth factor treatment, you should contact your hospital straight away.

CHOP chemotherapy
The most commonly used chemotherapy regimen for high-grade non-Hodgkin lymphoma is known as CHOP. This is made up of three intravenous drugs cyclophosphamide, hydroxydaunorubicin (often known as doxorubicin) and vincristine (Oncovin) and oral prednisolone tablets. You usually dont need to stay in hospital to have this treatment. The intravenous drugs are given on the first day of each cycle and you will be given the prednisolone tablets to take at home for the first 5 days of each cycle. This is followed by a rest period. Traditionally cycles of CHOP have been given every 3 weeks. Recently some doctors have used G-CSF to speed the recovery of the bone marrow and have given the cycles every 2 weeks. This is known as CHOP14; the 3-weekly schedule is CHOP21. For many people with high-grade lymphoma (not T-cell lymphomas) a drug called rituximab (MabThera) is
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Treatment of high-grade non-Hodgkin lymphoma

added to the CHOP . The treatment is then known as R-CHOP . Rituximab is an antibody therapy. It is given intravenously, usually on the first day of each cycle. Rituximab therapy is described in more detail on pages 46 47 .

Other chemotherapy regimens


There are many other chemotherapy regimens used in the treatment of high-grade lymphoma. It would be impossible to describe all of these regimens in this booklet. We have described CHOP in more detail because it is given at some stage to many people with high-grade lymphoma. You should be aware that if you are receiving a different treatment it may be quite unlike CHOP chemotherapy. Your hospital team will give you full information about exactly how your treatment will be given. They will also be able to tell you what side effects you should expect from the drugs being used. Do ask your team if you are unsure about anything to do with your treatment. We have more information about the different types of chemotherapy. If you would like to talk to someone about your chemotherapy please ring our helpline (0808 808 5555).

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High-grade non-Hodgkin lymphoma

Antibody therapy
Antibody therapies are often used in the treatment of high-grade non-Hodgkin lymphoma. They are usually used in combination with chemotherapy.

What is antibody therapy?


All cells have special proteins on their surface, called antigens. An antibody is a protein that recognises and can bind to a specific antigen. Monoclonal antibodies are proteins that have been specifically made to recognise an antigen that is more common on the surface of lymphoma cells than normal cells. When the antibody recognises the antigen on the lymphoma cell, it attaches itself to the antigen. This helps the lymphoma cell to die or helps your immune system to destroy it. Antibody therapy (sometimes called immunotherapy) is different from chemotherapy and radiotherapy because it targets cancer cells more specifically. This means it does not have the same side effects as chemotherapy or radiotherapy. There are now many antibodies in use for different kinds of cancer. The monoclonal antibody most often used for lymphoma targets the CD20 antigen. The treatment is called rituximab (or MabThera). Because CD20 is found on B lymphocytes only, rituximab is used only for B-cell lymphomas. New antibody therapies are in development and these will target different antigens.

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Treatment of high-grade non-Hodgkin lymphoma

How is antibody therapy given?


Rituximab for high-grade non-Hodgkin lymphoma is given in combination with chemotherapy. It is usually given at the start of each cycle of treatment. Rituximab is given as an infusion (drip) in the same way as other intravenous drugs. The first infusion is given quite slowly to help prevent side effects. It may take a few hours. Further infusions may be given more quickly (often over 60 90 minutes) if you have not had major side effects. Rituximab is usually given as an outpatient, so you dont normally have to stay in hospital.

Side effects of antibody therapy


The side effects of antibody therapy usually occur while the drug is being given rather than afterwards. The commonest side effects are shivers, fevers and flulike symptoms. These are more common with the first infusion, which is why the first one is given more slowly. The side effects are usually easy to treat. You will also be given drugs to prevent side effects before the infusion is started. Occasionally people have more serious side effects caused by an allergic reaction. If this happens, you may need to stay in hospital to be monitored for a while. It may be possible to give the infusion more slowly in future or your doctor may decide it is best to avoid further antibody therapy. For more information about rituximab please ring our helpline (0808 808 5555).
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High-grade non-Hodgkin lymphoma

Radiotherapy
What is radiotherapy?
Radiotherapy uses high-energy X-rays, similar to those used to take an X-ray but given in much higher doses. The X-rays are directed to precise areas and can kill off cancer cells by stopping them dividing. Radiotherapy is used for some people with high-grade non-Hodgkin lymphoma. Lymphoma cells are very sensitive to radiotherapy but the treatment can only be given to small areas. It may sometimes be used on its own as a treatment for early-stage disease. More often, it is used in combination with chemotherapy.

How is radiotherapy given?


Radiotherapy is usually broken up into a series of sessions known as fractions. These are usually given daily, Monday to Friday, and you can go home after each treatment. The number of fractions will vary but treatment is often spread over a period of weeks. Radiotherapy is only available in certain specialist centres so you may need to travel some distance for your treatment. For this part of your treatment your care will be led by a clinical oncologist (radiotherapist). They will meet with you to discuss your treatment beforehand. A radiotherapy department can feel like quite a high-tech and impersonal environment, with big machines and no windows. It can be unnerving at first, particularly as you will be left alone for some of the time. The staff will do their best to make you feel comfortable, so do let them know if you have any particular concerns or worries.
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Treatment of high-grade non-Hodgkin lymphoma

The first stage of treatment is planning. Radiographers will first take a special scan of the area to be treated. A computer then produces a precise map of your lymphoma. This is used to plan where your radiotherapy should go. The radiographer will mark some dots on your skin with a type of marker pen. They may also ask you to have a couple of more permanent, tiny marks on your skin. The marks are used to line your treatment up precisely each time so need to remain visible. If you are having radiotherapy to the head and neck, a shell will be made, usually from a plastic mesh. This will keep your head still and in the correct position. It also means you will not need markings on your skin. During treatment you will be carefully positioned on a narrow table and asked to stay very still. The radiographer may cover certain parts of your body that do not need treatment. The lights in the room are dimmed while the machine is lined up, then the lights are turned back up. Your treatment will take between 5 and 20 minutes and is painless. You will be left alone during this time but the radiographer will be in the room next door. They can hear you and will be able to speak to you. This kind of radiotherapy does not make you radioactive. There will be no risk to those close to you. Although the radiotherapy itself is completely painless, it can have uncomfortable side effects.
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High-grade non-Hodgkin lymphoma

What are the side effects of radiotherapy?


The side effects of radiotherapy will depend on what part of your body is being treated. You will be given information about what to expect and how to take care of yourself. You may find that you have no side effects to start with but that they become more obvious towards the end of your course of treatment. Most treatment side effects are short term. Some may be long term or permanent. Your doctors should discuss this with you before treatment starts. If you would like to talk about the side effects of treatment ring our helpline (0808 808 5555). It is important to let your hospital team know about your side effects. Tell them if any of the side effects change during your treatment. The radiographers wont always ask how you are feeling. If you dont say anything, they might assume you are OK. There are usually things that can be done to help with side effects. The following are some of the commoner side effects of radiotherapy. It is not possible to discuss every side effect in this booklet. Do discuss with your hospital team if you have concerns about side effects not mentioned here. You will find some tips for coping with these side effects in the appendix at the end of this booklet.

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Treatment of high-grade non-Hodgkin lymphoma

Fatigue Fatigue is one of the most common experiences for people with lymphoma. Fatigue is also a common side effect of radiotherapy. It may be some months after treatment before your fatigue goes away. Sore skin The skin in your radiotherapy treatment area may become pink, dry and itchy. If you have dark skin it might become darker. Rarely the skin can blister, a bit like sunburn. This is more likely to happen in folds of skin such as under the breast or in the groin. Skin reactions are usually at their worst a few days after the end of treatment and then start to heal. You will be given instructions about caring for your skin. You may be asked to avoid bathing the area. Hair loss If you are having radiotherapy, hair loss should only occur in the area being treated. This hair loss will usually only be temporary. Your hair should start to grow back a few months after treatment. Risk of infections and anaemia Your blood cell count may be affected by radiotherapy, particularly if any large bones are in the area being treated. This may cause a low white blood cell count (sometimes known as neutropenia). This can make you more at risk of infection during treatment. It can also cause anaemia (a shortage of red blood cells). This can add to feelings of fatigue and also make you feel short of breath.
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High-grade non-Hodgkin lymphoma

Sore mouth and difficulty swallowing If you are having radiotherapy to the head, neck or upper chest, you may find that your mouth or throat becomes sore. You may also find that food starts to taste different or metallic.

If the area of the radiotherapy includes your salivary glands, your mouth will also become very dry. Your doctors may recommend artificial saliva or drugs to increase the amount of saliva you produce to help with this. It may take several months for this side effect to improve and sometimes it may be permanent. If you are having radiotherapy to the chest or neck, you may also find that swallowing becomes difficult. Feeling sick Sometimes radiotherapy can make you feel sick, in particular if your abdominal area is being treated. If you are feeling sick, tell the radiographers. It may help to have anti-sickness medications (called antiemetics) before each treatment starts. If you are taking anti-sickness drugs that arent working, tell the radiographers. Sometimes a change in the anti-sickness drug is needed.

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Treatment of high-grade non-Hodgkin lymphoma

Stem cell transplants


Some people with high-grade non-Hodgkin lymphoma need high doses of treatment to manage their disease. These people might include: people with relapsed disease, meaning the lymphoma has come back after their first treatment people who are considered to be at high risk of relapse people whose disease has not responded to the standard treatment people involved in a particular clinical trial. Many treatments for lymphoma cause temporary damage to the cells of your bone marrow, which make the cells of your blood. Sometimes very high doses of chemotherapy and/or radiotherapy may be needed to kill all of your lymphoma cells. Such doses are likely to cause permanent damage to the bone marrow, but can be safely given if your bone marrow is given help to recover afterwards. This help comes in the form of a transplant (or transfusion) of stem cells. These cells may be collected either from a suitable donor or from you before you receive the high-dose treatment. After the stem cells have been infused, they find their way into your bone marrow. There, they start to grow and produce new blood cells. A transplant can involve using your own stem cells (an autologous transplant) or someone elses stem cells (an allogeneic transplant). In most cases, stem cell transplants for high-grade non-Hodgkin lymphoma will
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use your own stem cells. These are typically taken part way through your treatment and frozen until they are needed. The high-dose therapy can cause a number of side effects. These will vary depending on the exact treatment being given. Most are the same as those already discussed in the chemotherapy or radiotherapy sections but they may be more severe. During the recovery period you will be very prone to infections. You will find more on the side effects of chemotherapy on pages 35 42 and on the side effects of radiotherapy on pages 51 52 Most people have to stay in hospital for their stem cell transplant. This can be 3 6 weeks in all. Some hospitals do offer part of the treatment as an outpatient if you are well enough. Your hospital team will discuss what to expect from the procedure and the possible side effects before you have your transplant. We produce two booklets about stem cell transplants for lymphoma, one on autologous transplants, the other on allogeneic transplants. If you would like a copy of either of these please ring our helpline (0808 808 5555).

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Treatment of high-grade non-Hodgkin lymphoma

What happens after treatment?


Remission and cure
Remission means that the lymphoma has been controlled. There are different degrees of remission. A complete remission is where there is no visible evidence of the lymphoma following treatment. A partial remission is where the lymphoma has been reduced by at least a half. Specialists treating high-grade non-Hodgkin lymphoma will aim for a long-term complete remission. You may find that your specialist prefers to say you are in remission rather than you are cured. This is because they cannot say for certain whether or not your disease will come back. But the longer you have been in remission, the less likely it is that your disease will come back.

Follow-up appointments
You will see your specialist or another member of the hospital team regularly once your treatment has finished. These follow-up appointments are to see how you are feeling, to monitor your recovery from treatment and to check there are no signs of the lymphoma coming back. You will have a brief physical examination and you may have more blood tests. People who have had high-grade lymphoma will usually be followed up for 2 5 years after the end of their treatment. To start with, your appointments will be frequent, possibly once a month. If you stay well, the appointments will be spread out to every 6 months and eventually to only once a year.
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High-grade non-Hodgkin lymphoma

If you are worried about your health at any time, you dont have to wait for your next appointment. You should contact your GP or hospital team to discuss your concerns.

Most people worry about their follow-up appointments and you might not want to go back to be seen. But these appointments are an important part of your care. They give you a chance to talk about anything that might be on your mind. It can help to write these things down when you think of them and take the list with you. For more information about follow-up after treatment please ring our helpline (0808 808 5555).

What happens if my lymphoma comes back?


In some cases, lymphoma can come back following the initial course of treatment. This is called a relapse. Relapse of high-grade non-Hodgkin lymphoma is more likely to happen within the first 2 years after treatment. If your lymphoma relapses, your specialist will want to repeat some of the tests you had at the start. It may be possible for you to have further treatment for relapsed high-grade non-Hodgkin lymphoma. This will be planned on the basis of your individual situation. It will depend on your overall health and what treatment you had before. Often a different type of chemotherapy can be offered. For some people, a stem cell transplant may be possible.

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Treatment of high-grade non-Hodgkin lymphoma

Summary
Your treatment will depend on the type and stage of your lymphoma as well as on your age and health.

Chemotherapy
Chemotherapy means drug treatment, usually with a combination of drugs in each treatment cycle. Treatment usually takes several months to complete. Chemotherapy is usually given into a vein (intravenous) and/or as a tablet (oral). It will most often be given to you as an outpatient There are many potential side effects of treatment. Your side effects will depend on what kind of treatment you are having. You should be given information about what side effects to expect. If you develop signs of infection or notice side effects, you should tell the team at the hospital immediately.

Antibody therapy
Antibody therapy is given with chemotherapy for some high-grade non-Hodgkin lymphomas. It works by attaching itself to a protein on the lymphoma cell. It helps the cell to die or helps your immune system to destroy it. The antibody therapy most commonly used for non-Hodgkin lymphoma is rituximab (MabThera). Antibody therapy is given as an intravenous infusion. It will normally be given to you as an outpatient.
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High-grade non-Hodgkin lymphoma

Flu-like symptoms during the infusion are the commonest side effect. You will be given drugs to prevent these.

Radiotherapy

Radiotherapy for high-grade non-Hodgkin lymphoma is most often used along with chemotherapy. Radiotherapy is painless. Each fraction of treatment, usually given Monday to Friday, takes between 5 and 20 minutes to give. It usually continues for several weeks. The side effects of radiotherapy depend on the part of your body that is being treated. They tend to develop towards the end of treatment. You should be given information about what side effects to expect. If you notice any side effects, you should tell the team at the hospital as there are often things that can be done to help.

After treatment
You will have check-ups for some years after your treatment has finished. These will be frequent to start with, gradually reducing to once every 6 12 months. If you are worried about anything, you dont have to wait until your next appointment. Contact the clinic or your GP to talk about your concerns. Sometimes lymphoma can relapse after initial treatment. Treatment for relapsed lymphoma will depend on your individual situation.
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Types of high-grade non-Hodgkin lymphoma


B-cell non-Hodgkin lymphomas T-cell non-Hodgkin lymphomas Non-Hodgkin lymphomas associated with immunodeficiency states

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High-grade non-Hodgkin lymphoma

This part of the booklet discusses many of the different types of high-grade lymphoma in more detail. We would suggest that, at least at first, you read only the section on your particular type of high-grade lymphoma. You will find the individual types of lymphoma we describe in this section listed in the contents on page 5. If you are not sure exactly what kind of lymphoma you have, we would suggest you check this with your specialist. It may be confusing or distressing to read about illnesses that are not relevant to you. We have not been able to describe every type of highgrade non-Hodgkin lymphoma. If you have been told you have a type of lymphoma that you do not see listed in this booklet, you may wish to check with your specialist. Your specialist may have used an alternative name or they might be able to tell you if your lymphoma is similar to another type that is described here. For each lymphoma we are aiming to answer the following questions: What does the name mean? Who typically gets it? How might it affect me? How might it be treated?

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Types of high-grade non-Hodgkin lymphoma

B-cell non-Hodgkin lymphomas


Diffuse large B-cell lymphoma
What does it mean? Diffuse large B-cell lymphoma (DLBCL) is the most common kind of high-grade non-Hodgkin lymphoma. It causes almost 1 in 3 cases of non-Hodgkin lymphoma. The name describes the type of cell involved and its appearance. So it is a lymphoma formed from B cells that are large compared with the cells seen in other lymphomas. The cells have a diffuse pattern, meaning they are spread throughout the lymph node and have replaced its normal structure. Large lymphoma cells are likely to divide more quickly than smaller cells, so DLBCL is typically a fast-growing lymphoma. Who gets it? DLBCL is slightly more common in men than in women. It can develop in people of any age, but is more likely to occur in people aged over 50. Sometimes, DLBCL develops in people who have previously been found to have low-grade non-Hodgkin lymphoma. The low-grade lymphoma can change (or transform) into DLBCL. How will it affect me? Some people have no symptoms other than a lump or swelling. You may have more general symptoms such as weight loss, flu-like symptoms, night sweats or tiredness.
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Other symptoms of DLBCL will depend on what part of your body is involved. It is quite common for people with DLBCL to have disease in parts of the body other than the lymphatic system (extranodal disease). For example, it is relatively common for DLBCL to involve the bowel. This might cause symptoms such as abdominal pain and diarrhoea. Some people with DLBCL have disease that is stage I or II at the time of diagnosis. Most people will have more advanced disease (stage III or IV). You will find the stages of lymphoma described on pages 23 24. How will it be treated? The treatment of your DLBCL will depend on the stage of your disease, as well as your age and your general health. Most patients will receive a combination of chemotherapy and antibody therapy. The most commonly used regimen is R-CHOP . Radiotherapy is particularly good treatment for disease in a small area and is often used to treat large lymph nodes sometimes called bulky disease. Early-stage DLBCL can be treated with a limited number of cycles of chemotherapy (for example, 4 cycles of R-CHOP) followed by radiotherapy to the area of the enlarged lymph nodes. You will find R-CHOP described on pages 44 45.

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More advanced DLBCL will usually be treated with more cycles of chemotherapy (typically 6 cycles of R-CHOP). Radiotherapy is then usually only given if there were originally areas of bulky disease. Some people with DLBCL will also need treatment to the CNS (the brain and spinal cord). Spread to the CNS is more common when DLBCL occurs at certain sites, particularly extranodal sites. If lymphoma is found in your CNS, you may need to have other chemotherapy regimens instead of R-CHOP and/or intrathecal chemotherapy. Intrathecal chemotherapy is discussed in more detail on page 34. If there is currently no lymphoma in your CNS but your doctors think it may occur later, you may also have an additional treatment to reduce the risk of your lymphoma spreading to your CNS. This is known as CNS prophylaxis. For more information about preventive therapy for the central nervous system in lymphoma (CNS prophylaxis) please ring the helpline (0808 808 5555). Some people who have DLBCL that is not thought to have been cured after R-CHOP may be offered further treatment with a stem cell transplant.

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High-grade non-Hodgkin lymphoma

Burkitt lymphoma
What does it mean? Burkitt lymphoma (BL) is named after a doctor called Burkitt, who first wrote about this kind of tumour in children and young adults in Africa. The African-type (or endemic) Burkitt lymphoma that he described is related to infection with the EpsteinBarr virus (EBV). It has certain differences from the sporadic type of Burkitt lymphoma more often seen in the Western world. For instance, sporadic-type BL is less commonly related to EBV. EBV is linked to a number of different types of lymphoma. It appears to cause prolonged stimulation of normal B cells. This increases the chances of the cells becoming cancerous. Who gets it? BL affects adults, mainly at a relatively young age (average age 30). It also affects children (making up over a third of all lymphomas seen in children). It affects men more than women, and boys much more than girls. People who have HIV or AIDS are more likely to develop BL. In these cases, the disease develops partly because of the HIV and other viruses including EBV. Although BL also affects people who do not have HIV or AIDS, your doctors will probably recommend you have a test to rule out HIV infection as this is now a very treatable condition.
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How will it affect me? BL is a very fast-growing lymphoma. Your symptoms may have developed in a short space of time. It usually causes widespread enlargement of lymph nodes in different parts of the body, including the chest, the tonsils and the back of the nose and throat. BL can also be found in other organs such as the spleen and the liver. Many people will have disease in their bone marrow at the time they are diagnosed. You may also experience the other symptoms of lymphoma, including night sweats, tiredness, flu-like symptoms and unexplained weight loss. Compared with other lymphomas, BL is more likely to affect your bowel and the lymph nodes in your abdomen. People with BL very often go to the doctor with abdominal pain, nausea, vomiting and diarrhoea. The symptoms of BL may sometimes even be confused with appendicitis. It can also cause a collection of fluid within your abdomen (called ascites) or may cause your bowel to become obstructed or bleed. BL is more likely to involve your brain and spinal cord (the CNS) than other lymphomas. BL in children often affects the jaw. The disease commonly grows in the areas where permanent teeth are forming. How is it treated? Although BL is a very fast-growing lymphoma, it generally responds very well to an intensive combination
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of chemotherapy drugs. A commonly used combination is CODOX-M, which may be combined with IVAC. These initials refer to the names of the individual drugs. This type of chemotherapy can only be given to inpatients. CODOX-M on its own is used for people with earlystage BL and disease that has not yet had a big impact on general health.

CODOX-M/IVAC is used for people with more advancedstage BL. Often a large number of lymphoma cells are killed very quickly when this type of therapy is started. This can cause problems particularly with your kidneys, so you may be given a special drug to protect your kidneys. Rituximab is now added to chemotherapy for BL as this has been shown to improve the effectiveness of the treatment. You will find more information about rituximab on pages 4647 .

Other chemotherapy combinations may be used to control your BL if you are not fit enough for these treatments. Because BL commonly involves the CNS, all patients with BL receive treatment that is directed to their CNS. The chemotherapy regimens for BL use both those drugs that can enter the CNS from the bloodstream and drugs that are given intrathecally.
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Intrathecal chemotherapy is described in more detail on page 34. For more information on CNS lymphoma and its treatment please ring our helpline (0808 808 5555).

Chemotherapy for BL typically takes several months. Because intensive chemotherapy is needed to cure BL, you may get some quite severe side effects. To help you get through the treatment, you may need to stay in hospital for some of this period.

Mantle cell lymphoma


What does it mean? Mantle cell lymphoma (MCL) is an uncommon type of non-Hodgkin lymphoma. The word mantle is used because the lymphoma cells arise from a part of the lymph node called the mantle zone. The cells develop a particular genetic change that is useful for the diagnosis of this condition. This change results in uncontrolled cell growth and eventually the development of lymphoma. Although mantle cell lymphoma has the appearance of a low-grade (slow-growing) lymphoma under the microscope, its behaviour is variable. Some mantle cell lymphomas do grow slowly, but others grow more rapidly and require early and often intensive treatment.

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Who gets it? MCL makes up about 1 in 20 of all non-Hodgkin lymphomas. It is more common in men than it is in women. It is more likely to affect older people, typically those in their sixties. How will it affect me? Most people with MCL initially go to their doctor with enlarged lymph glands. You are most likely to have advanced-stage disease (stage III or IV) by the time it is diagnosed. MCL usually involves the bone marrow. The presence of lots of immature cancerous cells in the bone marrow can stop it making healthy blood cells. This can result in anaemia (a shortage of red blood cells). Anaemia causes shortness of breath, bone pain and fatigue. It can also result in thrombocytopenia (a shortage of platelets). Thrombocytopenia means you are more likely to bleed and that your body will be less able to stop any bleeding. The lymphoma cells can sometimes be found in the bloodstream too, which is similar to some kinds of chronic leukaemia. If there is also enlargement of your spleen without enlarged lymph nodes, it is likely your disease will be slow growing. It is very common for MCL to involve the bowel. This means you may have symptoms of bowel disease including diarrhoea and abdominal pain.

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How is it treated? MCL is difficult to treat successfully. It is not considered curable. Instead your doctors aim will be to get your disease into as good a remission as possible for as long as possible. A variety of treatments are possible and what is chosen for you will depend on your individual circumstances, for instance how fit you are. If you feel relatively well, you may have no active treatment until your symptoms become more difficult to live with. Delaying active treatment, especially if you have lymphoma cells in your blood and splenomegaly, will avoid side effects and keep the treatments until you need them. This is known as the watch and wait approach and is used in a number of low-grade lymphomas. When you do need treatment, as for other high-grade lymphomas, you will most likely receive a combination chemotherapy regimen. Your doctor will consider which of the treatments for MCL is best for you as there are a number of possibilities. Some are intensive chemotherapy treatments that may require you to be an inpatient. Rituximab is now added to chemotherapy for MCL as clinical trials have shown it improves results. Recent clinical trials have also suggested that giving rituximab after chemotherapy has been completed delays the return of the lymphoma in patients over the age of 60. This is known as maintenance rituximab therapy and is now starting to be used for MCL in most centres.

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Your doctors may also discuss the option of a stem cell transplant with you, which may give you a longer remission. But transplants involve major risks and side effects and are not suitable for everyone. Allogeneic transplants, where the stem cells come from another person, probably offer the only hope of cure in MCL. They are increasingly being used in people whose MCL has relapsed.

You will find more information about stem cell transplants on pages 53 54. Sometimes tablet chemotherapy, more commonly used for low-grade lymphoma, will be used to manage MCL. The drugs sometimes used in this way include chlorambucil (Leukeran), cyclophosphamide and fludarabine (Fludara). Clinical trials at present being done in MCL are testing drugs that are relatively new in lymphoma treatment. These include lenalidomide (Revlimid), bortezomib (Velcade), ofatumumab (Arzerra) and olaparib. These drugs kill lymphoma cells in various different ways. No one can say yet what the effects of these treatments will be. It is hoped they may be more effective than standard chemotherapy alone. You may at some stage be asked to take part in a clinical trial, but they are usually reserved for people who have already had chemotherapy.

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Primary mediastinal large B-cell lymphoma


What does it mean? Primary mediastinal large B-cell lymphoma (PMLBCL) is a type of diffuse large B-cell lymphoma (DLBCL) that particularly involves the lymph nodes in the mediastinum. The mediastinum is the area of the chest between the lungs. It contains the heart, the windpipe, the oesophagus (where food goes down) and other structures including lymph nodes, the thymus and major blood vessels. PMLBCL is believed to arise from B cells within the thymus. Who gets it? PMLBCL makes up around 1 in 50 of all cases of non-Hodgkin lymphoma. It affects younger people, typically those aged 20 40 years. It is twice as common in women as it is in men. How will it affect me? PMLBCL is quite often confined to the chest area at the time of diagnosis. The enlargement of the mediastinal lymph nodes can be quite dramatic. You may have a cough, shortness of breath and difficulty breathing. You may also experience the other symptoms of lymphoma including fever, night sweats and weight loss. How is it treated? PMLBCL is treated with a combination of chemotherapy and antibody therapy. The most commonly used treatment is R-CHOP. You will find R-CHOP described on pages 44 45.
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As the nodes in your chest may be very large, you may also have treatment with radiotherapy to the chest. The outcome of treatment is usually very good, generally even better than that seen in other types of DLBCL.

Primary central nervous system lymphoma

What does it mean? Non-Hodgkin lymphomas can affect almost any part of the body, including the brain and spinal cord, which is known as the central nervous system (CNS). Lymphoma can involve the CNS is a number of ways. A lymphoma that starts within and is confined to the CNS is known as a primary central nervous system lymphoma (PCNSL). Lymphoma can also spread to the CNS from some other part of the body (secondary CNS lymphoma) or press on the CNS from outside. Some cases of PCNSL involve the eye. This is known as primary intra-ocular lymphoma (PIOL). It can occur either on its own or at the same time as PCNSL. Who gets it? PCNSL is a very rare condition, making up less than 1 in every 100 non-Hodgkin lymphomas. It affects slightly more men than women. It is more common in those aged 50 70 years. It is also more common in people who have a suppressed immune system. It used to be common in people with HIV or AIDS but effective treatment for HIV means that there are now fewer cases.

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How will it affect me? PCNSL can affect a person in many different ways. Sometimes the lymphoma forms a mass that presses on part of the CNS. The exact symptoms it causes will depend on what part of the CNS is involved. Sometimes it will spread along the meninges (the layers of tissue that surround the CNS). You might hear this referred to as lymphomatous meningitis. Possible symptoms of PCNSL include headaches, changes in vision, drowsiness, problems with memory or balance, seizures, muscle weakness, change in personality or problems with speech. People with PCNSL are likely to become more confused and have worsening problems with understanding. For more information on CNS lymphoma please ring our helpline (0808 808 5555). How is it treated? PCNSL is a difficult disease to treat successfully. This is partly because people with PCNSL are often very sick by the time the lymphoma is found. This means treatment will need to be planned on an individual basis. Most people will have treatment with steroids, often the drug dexamethasone is chosen. The steroids can make the lymphoma shrink, at least for a while, and help to reduce the swelling of the brain around the lymphoma. This can help to relieve symptoms in the short term. It may also help to make people fitter so that they can then have other treatments.

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If you are fit enough, you will probably be offered chemotherapy. Chemotherapy for PCNSL involves high doses of the few drugs that are able to reach the CNS from the bloodstream (usually methotrexate together with cytarabine). These are given intravenously as an infusion (drip) and you will need to be an inpatient. Radiotherapy to the brain may also be used for PCNSL. It is not likely to cure the disease on its own, so may be given after chemotherapy or as a palliative treatment. Large doses of radiotherapy may not be suitable for everyone, in particular people who already have symptoms such as confusion or problems with understanding. These symptoms and others such as memory loss can sometimes worsen or even occur for the first time following radiotherapy. This is most likely to happen if you are more than 60 years old or have had previous chemotherapy. Your consultant should fully discuss these risks with you before you start treatment. Occasionally people with PCNSL may be considered for high-dose therapy and a stem cell transplant. This will be suitable for only a small number of people, generally those who are younger, fitter and have done well with earlier therapy. PIOL may be treated in a similar way to PCNSL. Clinical trials continue to test which treatments are best for people with PCNSL and PIOL.

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T-cell non-Hodgkin lymphomas


Lymphoblastic lymphoma
What does it mean? Lymphoblastic lymphoma (LBL) grows from an immature lymphocyte known as a lymphoblast. This is a type of lymphocyte that is at an early stage of development. It is sometimes just called a blast. LBLs are closely related to acute leukaemia. Both are diseases of immature blood cells. In theory, lymphoma is a disease that forms lumps in the lymphatic system, and leukaemia is a disease that grows in the bone marrow and the blood. In practice, the two diseases often have the same effects on the body. Like leukaemia, LBL can result in lots of immature cells in the bone marrow and blood. LBL can grow from either B or T cells. T-cell LBL is more common than B-cell LBL. Who gets it? LBL affects young people, typically those in their late teens or twenties. It is more common in men than women. How will it affect me? LBL grows very quickly. You may find that your symptoms develop within a short space of time. LBL very often causes a large tumour in the lymph nodes of the mediastinum. The mediastinum is the area of the chest between the lungs. It contains the
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heart, the windpipe, the oesophagus (where food goes down) and other structures including the major blood vessels and the thymus gland (where T cells mature). If the enlarged mediastinal nodes are big enough to squash the structures in your mediastinum, you may have a cough, shortness of breath or problems with your blood circulation. Large nodes such as these may be seen when you have a chest X-ray.

LBL can also damage the pleura (the layers surrounding your lungs). This can cause pain in your chest when you breathe. It sometimes causes a collection of fluid around your lung, known as a pleural effusion. You may also experience the other symptoms of lymphoma including night sweats, tiredness, flu-like symptoms and unexplained weight loss. As with leukaemia, problems may result from your bone marrow being involved. This is because the many immature cancerous cells in the bone marrow stop it making healthy blood cells. This can result in anaemia (a shortage of red blood cells). Anaemia causes shortness of breath, bone pain and fatigue. It can also result in thrombocytopenia (a shortage of platelets). Thrombocytopenia means you are more likely to bleed and that your body will be less able to stop any bleeding. LBL can also involve the CNS (the brain and spinal cord).

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How is it treated? LBL is treated with the same kind of chemotherapy as acute leukaemia. This treatment takes longer than most lymphoma treatments. It is divided into different phases. The first phase is called remission induction. This means that the treatment aims to get the lymphoma to a stage where it can no longer be easily detected. Remission induction involves having a number of different chemotherapy drugs given both intravenously in an infusion (drip) and orally. This usually happens over a period of several weeks, but the exact time will depend on your particular drug combination. You will have to stay in hospital for this time. The next phase is called consolidation. The aim of this phase is to kill off any lymphoma cells that are still floating around but that cant be easily detected. Consolidation involves more chemotherapy drugs given over a period of a few months. You usually dont have to stay in hospital during this time. The third phase of treatment is called maintenance. This involves mostly taking chemotherapy tablets but occasionally having more intravenous chemotherapy. Maintenance treatment is continued for 2 years. LBL can involve the CNS and even if you dont have lymphoma in your CNS now, your doctors will give you treatment to prevent it spreading there in the future (known as CNS prophylaxis). The CNS is treated by certain intravenous drugs included in the treatment regimen and by intrathecal chemotherapy.
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You will find more information about treating lymphoma in the CNS on page 34. For more information on CNS prophylaxis please ring our helpline (0808 808 5555). Some people may be offered high-dose therapy followed by a stem cell transplant when they are in remission. Whether this is recommended for you will depend on your individual situation. For more information on stem cell transplants please ring our helpline (0808 808 5555).

Peripheral T-cell lymphoma


What does it mean? Peripheral T-cell lymphoma refers to a group of similar lymphomas that all arise from T cells that start in the lymphatic system. The word peripheral means that the T cells mature in parts of the body peripheral to (or outside of) the thymus. Who gets it? Peripheral T-cell lymphoma makes up around 1 in 13 of all non-Hodgkin lymphomas. It is a disease that occurs in adults, more often in those aged over 50. It affects more men than women. How will it affect me? Most people will have advanced disease (stage III or IV) by the time they go to their doctor. Typically you will have enlarged, but relatively small, lymph nodes in many areas
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of your body. You may also have night sweats, fevers and unexplained weight loss. Enlargement of your liver and spleen may also occur and PTL often affects the bone marrow. Your skin may also be affected, causing reddened, itchy patches. How is it treated? Peripheral T-cell lymphoma is usually treated with chemotherapy, typically CHOP. In younger patients, the treatment may be intensified to include a stem cell transplant. You will find CHOP described on page 44. Peripheral T-cell lymphoma can be difficult to treat successfully with standard chemotherapy. If your disease does not respond to CHOP or you relapse after treatment, you may be offered other combinations of chemotherapy. One drug often given in this situation is gemcitabine (Gemzar). If you are otherwise fit, an allogeneic stem cell transplant may be another possibility. A number of new agents are becoming available and you may be asked to take part in a clinical trial.

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Anaplastic large cell lymphoma


What does it mean? Anaplastic large cell lymphoma (ALCL) is a type of T-cell lymphoma. The cells are large when viewed through a microscope and the word anaplastic means disordered growth. Experts have now divided ALCL into two different subtypes of lymphoma, depending on whether or not a particular type of protein is seen on the cells. The type with the protein has a change in the DNA of its cells that is different from other lymphomas. This change in the DNA creates a protein called ALK (anaplastic large-cell kinase). The cause of this change is unknown. Doctors refer to this type of lymphoma as ALK-positive ALCL . The other type is described as ALKnegative ALCL . It doesnt have the same DNA change and the ALK protein is not seen. Who gets it? ALCL makes up around 3 out of every 100 non-Hodgkin lymphomas. The ALK-positive type tends to affect younger adults or children and is more common in men and boys. ALK-negative ALCL is also more common in men but is seen in older adults, most commonly those between 40 and 65 years of age. How will it affect me? As well as affecting lymph nodes, ALK-positive ALCL often affects extranodal sites (other organs of the
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body), such as bone, skin and liver. You may also have other symptoms of lymphoma including fevers and weight loss. ALK-negative ALCL tends to be fast growing and can also sometimes involve extranodal sites such as the skin. In some cases, it is found only in the skin and nowhere else (known as primary cutaneous ALCL ). When it involves the skin, it causes patches that are reddened, itchy, raised and scaly. How is it treated? ALCL is often treated with CHOP chemotherapy, but ALK-negative ALCL usually responds less well to this. If you have ALK-negative ALCL and are fit enough, you may instead be offered more intensive treatment and possibly a stem cell transplant. You will find CHOP chemotherapy described in more detail on page 44. If you have particularly large lymph nodes, you may be offered treatment with radiotherapy too. New drugs, such as brentuximab (Adcetris), are being tested for possible use in the treatment of ALCL. They are being tried first where the lymphoma has come back after treatment or did not improve with treatment. If you have primary cutaneous ALK-negative ALCL (lymphoma confined to your skin), it may get better without treatment.

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Angioimmunoblastic T-cell lymphoma (AITL)


What does it mean? Angioimmunoblastic T-cell lymphoma (AITL) is a rare T-cell non-Hodgkin lymphoma. The term angio refers to blood vessels. The tumours of AITL are marked by the growth of abnormal blood vessels. AITL appears to be related to previous infection with the EpsteinBarr virus (EBV). The reason EBV infection causes this rare lymphoma in a few people remains unclear as yet. Who gets it? AITL makes up around 1 in every 100 non-Hodgkin lymphomas. It is more common in those aged over 60. It affects more men than women. How will it affect me? AITL can produce a wide range of symptoms. It is likely you will have widespread enlargement of your lymph nodes. You may also have enlargement of your liver and spleen. The other symptoms of lymphoma, especially fevers, itching, weight loss and night sweats, are also quite common in people with AITL. Unlike in many other lymphomas, the cancerous cells of AITL produce abnormal proteins. These proteins can cause your bodys immune system to react against your own cells. This is known as an autoimmune reaction, and can cause a rash, inflamed joints, pins and needles and numbness.
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How is it treated? AITL is difficult to treat successfully. The usual first treatment is CHOP chemotherapy. You will find CHOP chemotherapy described in more detail on page 44. It is common for AITL to relapse (come back) after treatment with CHOP . Other types of chemotherapy can be offered for relapsed disease. Some people may be offered treatment with a stem cell transplant. Stem cell transplants are discussed in more detail on pages 53 54. If you are not well enough for chemotherapy, you will probably have steroids to try and control your disease and improve your symptoms. Other treatments continue to be tested for AITL. One such treatment is the drug thalidomide (Thalidomide Celgene), which has proved to be useful in a number of different cancers. It is thought that thalidomide might help cancers die by stopping them growing the blood vessels they need to survive. At present this treatment, along with others, is still under investigation. You may be asked to take part in a clinical trial. We have a booklet about clinical trials. Please ring our helpline (0808 808 5555) for a copy.

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Enteropathy-associated T-cell lymphoma


What does it mean? Enteropathy-associated T-cell lymphoma (EATL) occurs in the small bowel (part of our intestines). It is also sometimes called enteropathy-type T-cell lymphoma or intestinal T-cell lymphoma. Enteropathy means a disease causing wasting or degeneration of the intestines, especially the small bowel. Who gets it? EATL is a rare disease. It makes up less than 1 in every 100 non-Hodgkin lymphomas. This disorder is thought to affect people with coeliac disease who may have been poorly controlled or not previously diagnosed. Coeliac disease is caused by sensitivity (meaning an abnormal reaction) to a protein called gluten that is found in wheat and other grains. Exposure to gluten results in wasting of the small bowel causing diarrhoea, abdominal pain and weight loss. EATL only happens in a tiny proportion of patients with coeliac disease. Some people with EATL might not know they have coeliac disease both may be diagnosed by the same tests on the bowel. Occasionally, EATL can also occur on its own, without the presence of coeliac disease. How will it affect me? EATL causes bowel and stomach problems, in particular you may have diarrhoea, abdominal pain and
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unexplained weight loss. It can also cause ulcers in the bowel and may occasionally cause the bowel to perforate. EATL can also cause enlargement of lymph nodes throughout the body. You may experience night sweats and fevers. Because it resembles a disease of the bowel, EATL can be difficult to diagnose. Other more common diseases are likely to be ruled out before lymphoma is considered. It can also be difficult to get good pictures of the bowel using standard scans. For these reasons, the disease may be present for some time before it is correctly diagnosed. How is it treated The usual treatment for EATL is CHOP chemotherapy. Coeliac disease is usually treated by taking a special gluten-free diet. EATL can be difficult to treat successfully. You may be offered other kinds of chemotherapy if CHOP fails to produce a response. Sometimes people with EATL will be offered high-dose therapy with a stem cell transplant. In some cases, people will have an operation to remove the affected parts of the bowel. This is not considered to be sufficient treatment on its own, as it is likely the lymphoma will come back in other areas. Surgery is usually carried out before chemotherapy.
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Adult T-cell leukaemia/lymphoma


What does it mean? Adult T-cell leukaemia/lymphoma (ATLL) is a rare disease that begins with the abnormal growth of a T cell. The terms leukaemia and lymphoma both describe cancers of the blood cells. The two conditions have a lot in common and can have similar effects on your body.

In theory, lymphoma is a disease that forms lumps in the lymphatic system and leukaemia is a disease that grows in the bone marrow and the blood. In practice, the two diseases overlap leukaemia can grow in the lymph nodes and lymphoma cells can occur in the blood and bone marrow. ATLL has features of both and can result in lots of cancerous T cells in the bone marrow and blood. Who gets it? ATLL is more common in people of Asian, African and Caribbean origin. The majority of cases occur in those aged 50 60 years. ATLL is one of the types of lymphoma associated with a specific virus, known as HTLV-1. The virus is particularly common in Japan, but is also common in South East Asia, the Caribbean, south-eastern USA and equatorial Africa. As a result, ATLL is also more common in these parts of the world. It is much less common in the UK.

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How will it affect me? The symptoms of ATLL are varied. Some people have no symptoms or notice just a lump. Other people will have more symptoms that come on rapidly such as lots of enlarged lymph nodes, night sweats, fevers and a rash. ATLL very commonly involves the bone marrow. This is why the term leukaemia forms part of the name. The presence of lots of immature cancerous cells in the bone marrow can stop it making healthy blood cells. This can result in anaemia (a shortage of red blood cells). Anaemia causes shortness of breath, bone pain and fatigue. It can also result in thrombocytopenia (a shortage of platelets). Thrombocytopenia means that you are more likely to bleed and that your body will be less able to stop any bleeding. ATLL commonly causes high levels of calcium in the blood (known as hypercalcaemia). This can result from disease in the bone. It can also happen because the lymphoma cells cause the body to produce abnormal hormones and enzymes. As well as enlarged lymph nodes, ATLL causes enlargement of the liver and spleen. It commonly involves the skin causing widespread reddened itchy patches. ATLL is also likely to affect the CNS (the brain and spinal cord), especially when it relapses.
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How is it treated? Treatments for ATLL will vary from person to person because the nature of the disease varies. People with the most slowly growing types who feel generally well will probably be offered antiviral treatments. These help to fight the HTLV-1 virus and can slow the progression of certain forms of ATLL. The drugs used include alpha-interferon, zidovudine (Retrovir ) and lamivudine (Epivir ; or both together as Combivir ). If your disease is growing more quickly and has more of the typical features of a lymphoma, you are more likely to have chemotherapy. The most common regimen is CHOP chemotherapy. Some people with ATLL who respond to chemotherapy initially will then be offered high-dose therapy and/or a stem cell transplant to reduce the risk of the disease relapsing. Clinical trials continue to investigate new treatments for ATLL. You may be invited to take part in a clinical trial.

Nasal-type NK/T-cell lymphoma


What does it mean? Nasal NK/T-cell lymphoma is a rare extranodal T-cell lymphoma. Extranodal means that it starts growing outside of the lymphatic system. NK refers to a white blood cell known as a natural killer cell, so-called because of the role it plays in your immune system. Cancerous NK cells are found in some cases of nasal-type lymphoma.
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Nasal lymphoma typically starts to grow in the sinuses, which are the air-filled spaces in the bones of your face that link to your nasal passages. Who gets it? This rare lymphoma occurs in people from Asia and Central America. Most patients with nasal-type NK/T-cell lymphoma appear to have had a previous infection with the EpsteinBarr virus (EBV). Exactly how the EBV infection is linked to this lymphoma is not yet clear. How will it affect me? Nasal lymphoma is most likely to be confined to the area around your nose. You may hear this referred to as stage IE or IIE, where the E stands for extranodal. The common symptoms of nasal lymphoma involve the nose, eyes, mouth or face. You may have a blocked nose, discharge or bleeding from your nose, weepy eyes, swelling of your face, difficulty swallowing or dental problems. In some people, the disease is found in other parts of the body. For example, it can involve the intestines or cause patchy skin rashes. How is it treated? Nasal NK/T-cell lymphoma can be difficult to treat successfully. It is usually treated with a combination of radiotherapy and chemotherapy to the areas affected by the lymphoma.
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Chemotherapy is used even if the disease seems to be confined to the nasal area. This is because lymphoma often comes back in places outside of the area treated with radiotherapy. Chemotherapy treats the cells that might be lurking elsewhere but which are difficult to detect. The CHOP chemotherapy regimen may be used, but often alternative chemotherapy regimens are used. You may be given L-asparaginase (Erwinase), a drug more often used in the treatment of childhood acute lymphoblastic leukaemia. It may be used with other drugs and has shown good activity in early trials.

Immunodeficiency-associated non-Hodgkin lymphomas


Immunodeficiency means that a persons immune system is weakened. This will make them more prone to infections but can also increase their risk of developing certain types of lymphoma.

Post-transplant lymphoproliferative disorder


What does it mean? Post-transplant lymphoproliferative disorders (PTLDs) are a group of disorders that affect people who have had a solid organ or allogeneic stem cell transplant. Typically there is proliferation (an increase) of certain lymphocytes. This is often related to EpsteinBarr virus (EBV) infection. Some of these disorders will take the form of a high-grade non-Hodgkin lymphoma, most often of B-cell type.
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Who gets it? PTLDs develop in people taking immunosuppressive drugs that affect the function of their T cells. People receiving immunosuppressive drugs following solidorgan transplants are 30 50 times more likely to develop non-Hodgkin lymphoma. How will it affect me? PTLD can cause of variety of symptoms. You may have an enlarged lymph node. Some people also get systemic symptoms such as weight loss, fever, night sweats and tiredness. Extranodal disease is relatively common. For instance your bowel, lungs, liver or CNS (brain and spinal cord) may be affected. If you have had a solid organ transplant, your transplanted organ may also be involved. This can sometimes be confused with rejection on initial tests. How is it treated? In some people, simply reducing or stopping immunosuppression (which allows the T cells to function again) may be enough to control the increased cells. This is most likely to work if you have only recently had your transplant. It does mean you will be more at risk of rejecting your transplant, so you will need to be very carefully monitored. If this is insufficient or if it is some time since you had your transplant, you may be treated with antibody therapy (rituximab). Sometimes this will be combined with chemotherapy, such as CHOP , that is more often used to treat high-grade lymphoma.
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HIV- and AIDS-related lymphoma


What does it mean? Lymphomas are more common in people who have something wrong with their immune system. This is particularly the case for people who have HIV infection or AIDS. People with HIV or AIDS are at risk of several different kinds of cancer, including several different lymphomas. The most common ones are diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma. Sometimes people with HIV/AIDS will develop lymphoma that starts in and is confined to the brain and spinal cord. This is known as primary central nervous system lymphoma (PCNSL). Lymphoma in HIV/AIDS seems to result from prolonged high levels of stimulation of B cells. This may happen as a result of the presence of viruses. Who gets it? Anyone with HIV infection can develop non-Hodgkin lymphoma, the likelihood of this and the type of lymphoma will depend on how well the HIV is controlled, for example the level of the CD4 count and the viral load. The introduction of effective treatment for AIDS, known as cART (combined anti-retroviral therapy), has led to a dramatic fall in the number of people developing lymphoma. In particular PCNSL is now much less common.
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How will it affect me? How your lymphoma affects you will depend on what kind of lymphoma you have. You should refer to the information on your particular type of lymphoma elsewhere in this part. How is it treated? What treatment you have will depend on what kind of lymphoma you have. The treatments for each lymphoma can be found elsewhere in this part. Most likely you will be given the same treatment as someone with lymphoma who does not have HIV infection. It is very important though that you have joint care, which means your HIV team work closely with your lymphoma team. This can help to reduce the number of specific complications you may face. Since the introduction of cART, the treatment of AIDS-related lymphomas has been increasingly successful. The results of chemotherapy may now be similar to those seen in people without HIV infection but extra care is needed during treatment. People with AIDS already have a damaged immune system. Treatments for lymphoma will cause extra damage to healthy immune cells, so the risks of infection are further increased. If you are taking cART, you should continue this alongside your chemotherapy. You should also continue to take PCP (pneumocystis pneumonia) prevention and other preventative medicines during chemotherapy.

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Conclusion
Whatever your situation, we hope that this booklet has helped you to understand more about high-grade non-Hodgkin lymphoma, particularly the type that is most relevant to you, and the sort of treatments your doctors are likely to recommend.

If you would like further information about anything mentioned in this booklet, or if you have any concerns that you would like to discuss, please get in touch with us on our free confidential helpline (0808 808 5555) or email information@lymphomas.org.uk

How you can help us We continually strive to improve our information resources for people affected by lymphoma and we would be interested in any feedback you might have on this booklet. Please visit www.lymphomas.org.uk/ feedback or email publications@lymphomas.org.uk if you have any comments. Alternatively please phone our helpline on 0808 808 5555.
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Glossary

Glossary
AIDS acquired immune deficiency syndrome (AIDS) is a disease caused by HIV (human immunodeficiency virus), which stops the immune system fighting off or preventing infection the use of someone elses tissue (eg stem cells) shortage of red blood cells in the blood drugs given to make a part of the body numb (a local anaesthetic) or the whole body numb (a general anaesthetic) a protein that kills off disease-causing cells or organisms such as bacteria the use of a persons own tissue (eg stem cells) small organisms, some of which can cause disease a test that takes some cells to be looked at under a microscope a blood test that counts the different types of cells in your blood including the red blood cells, the different types of white blood cells, and platelets

Allogeneic Anaemia Anaesthetic

Antibody Autologous Bacteria Biopsy Blood count

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Bone marrow

spongy material at the centre of long bones that produces the bodys blood cells something that is long term and doesnt go away completely the central nervous system (CNS) consists of the brain and spinal cord a computerised tomography (CT) scan uses X-rays to give a picture of the inside of your body in slices a block of chemotherapy that is followed by a rest period to allow the normal healthy cells to recover naming a condition or disease the layer of muscle that lies below the lungs; divides your body between your chest and abdomen which stands for deoxyribonucleic acid, is the hereditary material that makes up the genes within our cells present in a particular area or within a particular group of people (as opposed to sporadic) a doctor specialising in diseases of the blood and blood cells the study and description of diseased cells

Chronic CNS CT scan

Cycle

Diagnosis Diaphragm

DNA

Endemic

Haematologist Histology

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HIV

human immunodeficiency virus (HIV) is a virus that leads to a deficiency in part of the immune system

Immune system the parts of the body that fight off and prevent infection Immunodeficiency Intravenous Lymph Lymphatic vessels Lymphocyte weakness in or a breakdown of a persons ability to fight infections into a vein the clear fluid that carries material through the lymphatic system the tubes that carry lymph and connect with the lymph nodes a type of white blood cell that normally helps to fight infections caused by viruses; the cell that becomes cancerous in lymphoma gland that acts like a sieve in the lymphatic system; involved in fighting infection cancerous; a tumour that can spread to other parts of the body a manufactured protein that may be used as targeted treatment to kill cancer cells magnetic resonance imaging (MRI) scan is a test using magnetic waves to give a picture of the body

Lymph node

Malignant Monoclonal antibody MRI

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Neutropenia Neutrophil

a shortage of neutrophils in the blood, which makes you prone to infection a type of white blood cell that is important in fighting infections caused by bacteria and fungi a doctor specialising in the treatment of cancer a person who examines diseased tissues a positron-emission tomography (PET) scan uses a radioactive form of sugar to look at how active cells are; often combined with a CT scan, it shows which bits of the body are affected by lymphoma the tiny fragments of cells in your blood that help form blood clots and stop any bleeding an estimate of the future course of a persons illness a person who takes X-rays or gives radiotherapy a doctor who can analyse X-rays and scans a doctor who specialises in radiotherapy (also known as a clinical oncologist)

Oncologist Pathologist PET scan

Platelets

Prognosis Radiographer Radiologist Radiotherapist

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Red blood cell

a cell that contains the pigment haemoglobin, which allows it to carry oxygen around our bodies a large organ found behind the stomach that is part of the immune system a term meaning that the spleen is of larger size than normal present in scattered or intermittent cases (as opposed to endemic) underneath the skin a change in the body or its functions that tells you something is abnormal a shortage of platelets in the blood, which makes you more prone to bleeding or bruising a collection of abnormal cells that are forming a lump a tiny organism that often causes disease a cell found in the blood and in many other tissues that helps our bodies to fight infections; several different types exist including lymphocytes and neutrophils

Spleen

Splenomegaly Sporadic Subcutaneous Symptom Thrombocytopenia Tumour Virus White blood cell

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Useful organisations
This is a short list of useful organisations, but there are many others. If you cant find the right organisation listed here, please ring our helpline (0808 808 5555). Benefit Enquiry Line Provides advice and information on a range of benefits to people with disabilities and their carers. 0800 882 200 www.gov.uk/browse/benefits/disability BEL -Customer-Services@dwp.gsi.gov.uk Jobcentre Plus Provides advice and information on benefits to people of working age. 0800 055 6688 www.gov.uk/browse/working/finding-job The GOV.UK website also includes a do-it-yourself benefits advice tool. British Association for Counselling and Psychotherapy Provides a list of accredited counsellors in your area. BACP House 15 St Johns Business Park Lutterworth Leicestershire LE17 4HB 01455 883316 www.bacp.co.uk bacp@bacp.co.uk

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Useful organisations

Cancer Research UK Offers information and statistics on all types of cancer, including treatment, prevention, screening and research. A team of specialist cancer nurses can be contacted by phone by calling their freephone number. Angel Building 407 St John Street London EC1V 4AD 0808 800 4040 (MondayFriday, 9am 5pm) www.cancerhelp.org.uk via website Carers UK Provides free and confidential information for carers. 20 Great Dover Street London SE1 4LX 0808 808 7777 www.carersuk.org via website Depression Alliance Provides information about the symptoms and treatment of depression, national and local groups. 20 Great Dover Street London SE1 4LX 0845 123 2320 www.depressionalliance.org information@depressionalliance.org

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Infertility Network UK Provides information and emotional support to those affected by infertility. Charter House 43 St Leonards Road Bexhill-on-Sea East Sussex TN40 1JA 0800 008 7464 www.infertilitynetworkuk.com via website Leukaemia & Lymphoma Research Funds research into the causes and treatment of leukaemia, lymphoma and related diseases. 3940 Eagle Street London WC1R 4TH 020 7504 2200 www.leukaemialymphomaresearch.org.uk info@beatingbloodcancers.org.uk Macmillan Cancer Support Provides practical, medical, emotional and financial support to those living with cancer. 89 Albert Embankment London SE1 7UQ 0808 808 0000 www.macmillan.org.uk via website

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Useful organisations

NHS Choices An information website which aims to help you make choices about your healthcare. www.nhs.uk NHS Direct Provides advice and health information 24 hours a day via the internet and telephone. For England and Wales 0845 4647 www.nhsdirect.nhs.uk www.nhsdirect.wales.nhs.uk/ For Scotland (NHS 24) 08454 242424 www.nhs24.com For Northern Ireland (Health and Social Care in Northern Ireland) www.n-i.nhs.uk Terence Higgins Trust The largest provider of information, support and education about HIV and AIDS. Branches nationwide 0808 802 1221 www.tht.org.uk

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Selected references
The full list of references is available on request. Please contact us via email (publications@lymphomas.org.uk) or telephone 01296 619409 if you would like a copy.

Books
Swerdlow SH, et al (eds). WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. 2008. IARC, Lyon. Hatton C, et al. Lymphoma. 2008. Health Press: Oxford.

Journal articles
Kaplan LD. HIV-associated lymphoma. Best Practice and Research in Clinical Haematology, 2012. 25:101107 . Phillips A, et al. An update on the management of peripheral T-cell lymphoma and emerging treatment options. Journal of Blood Medicine, 2011. 2: 119 129. Schmitz N, et al. Treatment and prognosis of mature T-cell and NK-cell lymphoma: an analysis of patients with T-cell lymphoma treated in studies of the German High-Grade Non-Hodgkin Lymphoma Study Group. Blood, 2010. 116: 3418 3425. Atmar J. Review of the safety and feasibility of rapid infusion of rituximab. Journal of Oncology Practice, 2010. 6: 91 93. Parker A, et al. Management of post-transplant lymphoproliferative disorders in adult solid organ transplant recipients: BCSH and BTS guidelines. British Journal of Haematology, 2010. 149: 693 705. Ferreri AJ. How I treat primary CNS lymphoma. Blood, 2011. 118: 510 522. Halfdanarson TR, et al. Hematologic manifestations of celiac disease. Blood, 2007 . 109: 412 421.

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Selected references

Weiss R, et al. Acquired immunodeficiency syndromerelated lymphoma: simultaneous treatment with combined cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy and highly active antiretroviral therapy is safe and improves survival-results of the German Multicenter Trial. Cancer, 2006. 106: 1560 1568.

Other sources
National Institute for Health and Clinical Excellence (NICE) 2003. Improving Outcomes in Haematological Cancers: The Manual. Available at: guidance.nice.org.uk/CSGHO (accessed March 2012). National Institute for Health and Clinical Excellence (NICE) September 2003. Rituximab for aggressive non-Hodgkins lymphoma. Available at: guidance.nice.org.uk/TA65 (accessed March 2012). British Committee for Standards in Haematology (BCSH) April 2010. Best Practice in Lymphoma Diagnosis and Reporting. www.bcshguidelines.com/documents/Lymphoma_diagnosis_ bcsh_042010.pdf (accessed March 2012). Cancer Research UK. Non-Hodgkin lymphomas UK incidence statistics 2009. Available at: info.cancerresearchuk.org/cancerstats/ types/nhl/ (accessed July 2012).

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Appendix: Tips for coping with side effects


The following information suggests possible ways of dealing with side effects. This is only introductory information be sure to ask your medical team for advice about dealing with your side effects. Generally speaking you should tell your team if you feel unwell in any way. For more information about dealing with specific side effects please call us on 0808 808 5555. Low white cell count (neutropenia) Contact the hospital immediately if you develop signs of infection such as: fever temperature above 38C chills and sweating mouth sores and ulcers cough or sore throat redness or swelling around sores on the skin diarrhoea burning sensation when passing urine unusual vaginal discharge or itching feeling generally unwell or disorientated. Wash well and regularly. Wash hands before meals, after using the toilet, after using public facilities.

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Appendix: Tips for coping with side effects

Avoid places where infection risk is increased, such as swimming pools. Stay away from people with infections such as colds, flu, chickenpox or diarrhoea and vomiting, if possible. Stay away from crowds, eg avoid cinemas and busy shops. Avoid foods that contain lots of live bacteria. These include: soft cheeses takeaway food raw or undercooked eggs ground pepper undercooked meats and fish pat. Dont eat anything that is past its sell-by date and consume refrigerated food within 24 hours once it has been opened. Take care when handling pets avoid bites or scratches and wash your hands afterwards. If possible, get someone else to deal with litter trays and faeces. Wear gloves for gardening. Low red cell count (anaemia) Tell your doctor if you feel short of breath, abnormally tired or have abnormal aches and pains. Ask about what treatment you could have for anaemia.

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Low platelet count (thrombocytopenia) Report bruising or bleeding. Contact the hospital immediately if you feel very unwell, faint or clammy. Avoid contact sports or very vigorous exercise. Take care to avoid injury when doing day-to-day things like cooking and gardening. Change in taste and loss of appetite Try to eat little and often and avoid big meals. Eat whenever you are hungry, whether or not this is your usual mealtime. Avoid things you dont like. Try foods that taste stronger marinated foods, savoury rather than sweet. Allow food to cool a little before eating it warm rather than hot. Have a ready supply of things that are quick and easy to prepare. Try to supplement your diet with nutritious drinks, but not at mealtimes. Drinking through a straw may be helpful. Eat with others, preferably in a pleasant environment. Take exercise where possible. Follow the mouth-care advice youve been given, such as rinsing your mouth before meals.

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Appendix: Tips for coping with side effects

Constipation Ask your doctor if your treatment might cause constipation and ask for advice about using laxatives to prevent it. Make sure you drink plenty. Try a hot drink in the mornings. Eat a high-fibre diet if possible. Take gentle exercise. Fatigue Tell your doctor or nurse. Take regular light exercise, such as walking. Take regular short rests throughout the day. Ask your doctor if you are anaemic and whether any treatment will help your anaemia. Ask if any of your medicines may be causing fatigue and if these can be changed or stopped. Plan your activities: dont worry about the less important things and plan the important things for when you have more energy. Accept offers of help with day-to-day tasks. Aim for a good nights sleep on a regular basis. Eat well. Make time to see friends and take part in normal social activities.

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Feeling sick Take anti-sickness drugs. Tell one of your hospital team if they dont work. Try travel sickness wristbands from the pharmacy. These prevent nausea by using acupressure points. Try relaxation techniques. Avoid cooking smells and seek help with preparing meals. Eat smaller meals that are cold or at room temperature. Keep your surroundings as peaceful and clean as possible and encourage fresh air. Hair loss Have your hair cut short before your treatment starts. Discuss wigs with the hospital team, or try hats or scarves. Avoid using heated rollers or hairdryers; avoid chemical treatments such as perms and hair dyes. Avoid things that pull at your hair such as rollers and tight elastics. Use wide-toothed combs and soft-bristled hair brushes. Protect the skin of your scalp from becoming dry. Avoid exposure to heat and cold. Wearing a hairnet or towelling turban to bed will help to collect hair lost overnight. Use make-up, jewellery and accessories to give you more confidence.

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Appendix: Tips for coping with side effects

Peripheral neuropathy Tell your doctors or nurses if you have pins and needles or loss of feeling in your fingers or toes, loss of balance, abdominal pain or constipation. Take care to avoid injury to fingers and toes (they will be less sensitive than usual). Avoid extreme temperatures; keep your feet and hands warm as cold may make your symptoms worse. Wear gloves for gardening; and take care when cooking. Try gentle massage and exercise of fingers and toes by flexing and stretching for a few minutes four times a day. Wear comfortable shoes avoid high heels or shoes that are tight. Inspect your feet regularly to check for damaged skin in parts that are numb, particularly on the soles of your feet and around toenails. Reduced fertility Talk about fertility with your doctor before treatment starts. Discuss options for storage of sperm/embryos/eggs for use after treatment if necessary.

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Sore mouth, sore throat Visit your dentist before starting treatment. Practise good oral hygiene the hospital may prescribe special mouthwashes for you to use. Avoid mouthwashes containing salt or alcohol. Use a soft-bristled toothbrush. Rinse your mouth after meals. Keep your lips moist with lip creams. Avoid alcohol and tobacco. Avoid hot, spicy foods or foods that are coarse in texture. Try cool, easy to swallow things, like ice cream and yoghurt. Sipping drinks through a straw may help. Ask for painkillers or other medication to help. Sore skin Ask your team about looking after your skin. Do not use creams unless recommended by your specialist. Avoid soaps, talcum powder and deodorants. Avoid rubbing the skin. If bathing, use lukewarm water and pat dry with a towel. Men having radiotherapy to the head or neck should use electric razors rather than wet shaving, or avoid shaving altogether. Avoid exposure to sun and wind. Avoid swimming pools.
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When my husband was diagnosed with high-grade non-Hodgkin lymphoma, we were both in a state of shock. The clinical nurse specialist told us about the Lymphoma Association and using their services reduced that fear of the unknown. Their information is extremely helpful and easy to read, the helpline staff are wonderful, and being able to talk to others in the same position at a support group has been enormously helpful. Pat Aylett

High-grade non-Hodgkin lymphoma

This booklet will help you understand more about the main types of high-grade non-Hodgkin lymphoma. It describes how they typically affect people, as well as possible treatments and what can be expected. The Lymphoma Association provides specialist information and emotional support to anyone affected by lymphatic cancer. Get in touch today to see how we can help you.
Lymphoma Association PO Box 386, Aylesbury Bucks HP20 2GA General enquiries 01296 619400

Freephone helpline 0808 808 5555 information@lymphomas.org.uk www.lymphomas.org.uk


Lymphoma Association Registered charity no 1068395 LYM0012/HGNHLymphBk/2012ed4 4th edition produced 14.11.2012 Next revision due 14.11.2014 ISBN 978-0-9570044-0-5