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The information that follows is an overview of this type of cancer. It is based on the more detailed information in our document, Leukemia-Chronic Myeloid. This document and other information can be obtained by calling 1-800-227-2345 or visiting our Web site at www.cancer.org.
What is cancer?
The body is made up of trillions of living cells. Normal body cells grow, divide, and die in an orderly way. During the early years of a person's life, normal cells divide faster to allow the person to grow. After the person becomes an adult, most cells divide only to replace worn-out, damaged, or dying cells. Cancer begins when cells in a part of the body start to grow out of control. There are many kinds of cancer, but they all start because of this out-of-control growth of abnormal cells. Cancer cell growth is different from normal cell growth. Instead of dying, cancer cells keep on growing and form new cancer cells. These cancer cells can grow into (invade) other tissues, something that normal cells cannot do. Being able to grow out of control and invade other tissues are what makes a cell a cancer cell. In most cases the cancer cells form a tumor. But some cancers, like leukemia, rarely form tumors. Instead, these cancer cells are in the blood and bone marrow. When cancer cells get into the bloodstream or lymph vessels, they can travel to other parts of the body. There they begin to grow and form new tumors that replace normal tissue. This process is called metastasis (muh-tas-tuh-sis). No matter where a cancer may spread, it is always named for the place where it started. For instance, breast cancer that has spread to the liver is still called breast cancer, not liver cancer. Likewise, prostate cancer that has spread to the bone is called metastatic prostate cancer, not bone cancer.
Different types of cancer can behave very differently. For example, lung cancer and breast cancer are very different diseases. They grow at different rates and respond to different treatments. That is why people with cancer need treatment that is aimed at their own kind of cancer. Not all tumors are cancerous. Tumors that aren't cancer are called benign (be-nine). Benign tumors can cause problems they can grow very large and press on healthy organs and tissues. But they cannot grow into other tissues. Because of this, they also can't spread to other parts of the body (metastasize). These tumors are almost never life threatening.
Bone marrow
Bone marrow is the soft inner part of some bones like the skull, shoulder blades, ribs, pelvis, and backbones. Bone marrow is made up of blood-forming cells, fat cells, and tissues that help cells grow. The cells that make blood are called blood stem cells. These stem cells only make new blood-forming cells. They are different from embryonic stem cells because they cannot make any other kinds of cells. Stem cells divide to make new stem cells. But they also go through a series of changes to make blood cells. During this process they can become red blood cells, white blood cells, or platelets.
Platelets
Platelets aren't whole cells but rather are pieces of large cells that plug up holes in blood vessels caused by cuts or bruises. A person with a shortage of platelets can bruise or bleed easily.
Types of leukemia
Not all leukemias are the same. Leukemias are divided into 4 main types. Knowing the exact type of leukemia can help doctors better predict each patient's outlook (prognosis) and select the best treatment.
In acute leukemia, the cells grow fast but do not mature the way they should. The cells keep on splitting to form new cells and those cells build up in the bone marrow and blood. Without treatment, most patients with acute leukemia would live only a few months. Some types of acute leukemia respond well to treatment, and many patients can be cured. Other types of acute leukemia don't respond as well to treatment. In chronic leukemia the cells look mature but they are not really normal. The cells live too long, build up, and crowd out normal cells. Chronic leukemias tend to progress over a longer period of time, and most patients can live for many years. But chronic leukemias are often harder to cure than acute leukemias.
The information here is only about chronic myeloid leukemia (CML) in adults. The other types of adult leukemia are covered in different American Cancer Society documents. For information about leukemia in children, please see the document, Childhood Leukemia Overview.
There has been dramatic progress in treatment over the past several years. Most people with CML are now living at least 5 years after their cancer is found. The drugs used to treat CML today are still fairly new, so the average survival of people now being treated for CML is not known. To learn more, please see the section "Survival rates for chronic myeloid leukemia."
DNA changes
DNA is the chemical that carries our genes, the instructions for how our cells work. During the past few years, scientists have made great progress in learning how certain changes in DNA may cause normal bone marrow cells to become leukemia cells. Sometimes a person inherits DNA changes from a parent that greatly increase the risk of getting certain types of cancer. But these inherited changes are very rarely the cause of CML. Changes linked to CML usually take place during life rather than having been inherited before birth.
Chromosomes are long chain of DNA found in every cell. One chromosome change that has been linked to CML is called the Philadelphia chromosome. The Philadelphia chromosome is found in the leukemia cells of almost all patients with CML.
Genetic tests
Doctors may also use other very precise lab tests to diagnose and classify leukemia. You might hear some of the following terms used: cytochemistry, cytogenetics, FISH, and PCR. These tests are explained in our document Leukemia: Chronic Myeloid (Myelogenous).
Imaging tests
Imaging tests are ways of taking pictures of the inside of the body. They are not used to find leukemia, but they may be done to help figure out how much the disease has spread.
machine that surrounds the table. You might feel a bit confined while the pictures are being taken. Often before any pictures are taken, you may be asked to drink 1 to 2 pints of a liquid called oral contrast. This helps outline the intestine more clearly. You may also have an IV (intravenous) line through which you get a different kind of contrast dye. This helps better outline blood vessels and internal organs. The IV dye can cause some flushing (a feeling of warmth in the face or elsewhere). Some people get hives (itchy bumps). A few may have more serious allergic reactions like trouble breathing, feeling dizzy, or passing out. Before the scan, be sure to tell the doctor if you have ever had a reaction to any contrast material used for x-rays
Ultrasound
This test uses sound waves to make pictures of internal organs. Ultrasound can look for enlarged organs in the belly (abdomen). This is a very easy test to have done. For most scans, you lie on a table and a technician moves a small wand (transducer) over the part of the body being looked at, which is first smeared with a gel.
Chest x-ray
This is a plain x-ray of the chest. It isn't used to tell if someone has CML, but to look for lung problems.
Chronic phase
In this phase, less than 10% of the cells in the blood or bone marrow are blasts. Patients usually have rather mild symptoms (if any) and respond to standard treatments. Most patients are diagnosed in the chronic phase.
Accelerated phase
There are higher numbers of blasts (more than 10% but less than 20%) in the blood or bone marrow in this phase. These patients often have fever, poor appetite, and weight loss. They do not respond to treatment as well as during the chronic phase.
Very high or very low platelet counts Age 60 years or older Many chromosome changes in the CML cells Many of these factors are used in the Sokal system, which gives a score to predict outlook. It takes into account the person's age, the percent of blasts, the size of the spleen, the numbers of different kinds of cells, and other factors. Your doctor can tell you if any of these factors apply in your case and what they mean. In the past, the Sokal system and another one called the Euro score were used to divide patients into different risk groups. But it's not clear how useful these systems are now in helping to predict a person's outlook. Newer drugs like Gleevec, Sprycel, and Tasigna have led to major changes in the treatment and outlook of CML in recent years.
This section starts with general comments about the types of treatments used for chronic myeloid leukemia (CML). After that we outline treatment options based on the phase of the disease.
imatinib because of side effects or patients whose disease did not respond to imatinib. Later studies showed that they were better than imatinib for many patients with CML. They are now approved to be used as the first treatment for CML. These drugs are also taken as pills. They have many of the same side effects as imatinib, but can also have some different ones as well, so talk with your doctor about what you can expect. They are known to interact with other drugs, so be sure that your doctor is aware of any medicines you take, including over the counter medicines and supplements.
Interferon
Interferons are substances naturally made by some immune system cells. At one time interferon was one of the main treatments for chronic myeloid leukemia (CML), but the new targeted drugs, as described above, are now the mainstay of treatment. Interferon can be used to slow the growth of leukemia cells and is injected (given as a shot) under the skin every day for several years. Interferon can cause side effects. These include "flu-like" symptoms. Patients on this drug may also have problems with thinking and concentration. Side effects of interferon include: Muscle aches Bone pain Headaches Tiredness Nausea Vomiting Low blood cell counts Depression These problems usually get better once treatment is over. But some patients will have to stop treatment because of these side effects.
time is as part of the treatment during a stem cell transplant. It may also be used by itself later in the course of disease if other treatments have stopped working.
whole body, just before a stem cell transplant (see the section, "Bone marrow or peripheral blood stem cell transplant (SCT) for chronic myeloid leukemia"). The main short-term side effects of radiation treatment are sunburn-like changes in skin over the treated area, tiredness, and a higher risk of infection.
Bone marrow or peripheral blood stem cell transplant for chronic myeloid leukemia
Normal doses of chemotherapy (chemo) can harm normal cells as well as cancer cells. A stem cell transplant offers a way for doctors to use the very high doses of chemo needed to kill all the leukemia cells. Although the drugs destroy the patient's bone marrow, transplanted stem cells given after the chemo can restore the blood-making bone marrow stem cells. This is called a stem cell transplant (SCT). Stem cells for transplant are most often collected from the bone marrow or from the bloodstream (in a process called apheresis). Sometimes umbilical cord stem cells are used. Bone marrow transplant was more common in the past. Now it has been largely replaced by cells taken from the bloodstream (called peripheral blood stem cell transplant or PBSCT). These blood-forming stem cells can come from either the patient or from a donor whose tissue type closely matches that of the patient. The donor may be a brother or sister or less often a person not related to the patient.
After treatment, the stored stem cells are given to the patient into a vein like a blood transfusion. Then the waiting begins as the stem cells settle in the patient's bone marrow and start to grow and produce blood cells. People who get a donor's stem cells are given drugs to prevent rejection as well as other medicines to prevent infections. Usually within a couple of weeks after the stem cells are put in (infused) they begin making new white blood cells. Then they begin making platelets, and finally, red blood cells. Patients having SCT have to be kept away from germs (in protective isolation) as much as possible until their white blood cell count is at a safe level. They may be able to leave the hospital when their white blood cell count is near 1,000. Even after they go home, they will be seen in the outpatient clinic almost every day for many weeks.
"Mini-transplants"
Most patients over the age of 55 can't withstand a regular transplant that uses high doses of chemo. But some may be able to have what is called a "mini-transplant" (also called a non-myeloablative transplant or reduced-intensity transplant). In this type of transplant, patients get lower doses of chemo and radiation which do not destroy the all cells in their bone marrow. They then get the donor stem cells. These new cells enter the body and form a new immune system, which sees the leukemia cells as foreign and attacks them (called a "graft-versus-leukemia" effect). This approach is not often used in CML.
Bone damage, which can be so bad that part of the bone and joint must be replaced Graft-versus-host disease or GVHD is the main problem of a donor stem cell transplant. It happens when the immune system of the patient is taken over by that of the donor. The donor's immune system then begins reacting against the patient's other tissues and organs. Symptoms of GVHD can include weakness, dry mouth, nausea, severe skin rashes with itching, and severe diarrhea. The liver and lungs may also be damaged. The patient may also become tired and have aching muscles. If bad enough, GVHD can be fatal. Drugs that weaken the immune system are often given to try to keep GVHD under control.
Chronic phase
The standard treatment for chronic phase CML is either imatinib (Gleevec) or another targeted therapy drug like it. Most often, imatinib is the first drug tried. Some people in chronic phase may be treated with a stem cell transplant from a matched donor. While a stem cell transplant is the only treatment proven to cure CML, it does have some drawbacks. A matched donor must be found, and it doesn't work in all cases. It also involves the use of high-dose chemotherapy (chemo) and often radiation, which can cause severe side effects and can even be fatal.
Watching the patient's blood and bone marrow for a response is a very important part of treatment with a targeted therapy drug. Blood counts are watched closely usually about every 3 to 6 months for the first 2 years. About 7 out of 10 patients on imatinib will have a good response (normal blood counts and no signs of the Philadelphia chromosome) after about a year. This rate is even higher for patients on the other targeted drugs. But it's doesn't seem like these people are being cured. In studies, the CML often came back if the drug is stopped. For now, doctors recommend that people keep taking the drug. The goal of treatment with targeted therapy is a complete response. If this doesn't happen, or if the leukemia gets worse, there are several options. Treatment of CML if the first targeted drug doesnt work: Some patients stop responding to their drug treatment over time and a few don't respond at all. Options then could include: Increasing the dose of the targeted drug. Switching to one of the other targeted drugs (like from imatinib to dasatinib/Sprycel or nilotinib/Tasigna). This helps many people for whom the first drug is not working. For those who can't take these drugs or for whom they are not working, interferon or chemo may be an option. Stem cell transplant may be an option, especially for younger people who have a donor with a matching tissue type. Treatment of CML after a stem cell transplant: For some people who have a stem cell transplant, their leukemia doesnt go away completely. If they do not have graft-versushost disease (GVHD), doctors may try to get their new immune system to fight the leukemia. One way to do this is by slowly lowering the doses or stopping the immune suppressing drugs they are on. This is done very carefully to try to get an anti-leukemia effect without getting too much GVHD. Patients who do have GVHD after a stem cell transplant are often treated with imatinib or another targeted therapy drug.
Accelerated phase
During this phase, leukemia cells begin to build up in the body more quickly. Treatment choices depend on what treatments the patient has already had and are much like those used in the chronic phase. Higher doses of the targeted therapy drug being used may help. The patient may instead be switched to another targeted therapy drug. For the most part, patients in this phase are less likely to have a long remission with any treatment. About 2 out of 10 patients have some response to chemo, but it often lasts less than 6 months. A stem cell transplant may be the best option for most patients who are young enough. About 20% to 40% of patients in this phase are alive several years after a stem cell transplant. Most doctors prefer that the leukemia be in remission before starting the transplant procedure. To achieve this, chemo will often be used. In some cases, a stem cell transplant using the patient's own stem cells may be an option to try to get the CML back into the chronic phase, but it is not likely to result in a cure.
Blast phase
In this phase of CML, the leukemia cells are more abnormal. The disease acts more like an acute leukemia. For people with blast phase CML who haven't had treatment already, high-dose imatinib may be helpful. But it works in a smaller number of people and for a shorter length of time than when used earlier in the course of the disease. The newer targeted agents (dasatinib and nilotinib) may work better, but the disease still comes back after a while. Patients who respond to these drugs may want to think about a stem cell transplant, if possible. Most often, the leukemia cells act like cells of acute myeloid leukemia (AML), but often they don't respond to the chemo drugs used to treat AML. About 1 out of 5 patients will respond, but this response is usually short-lived. If this does occur, it may offer a chance to think about some type of stem cell transplant. A small number of patients in this phase have blast cells that look like those of acute lymphocytic leukemia (ALL). These cells respond better to chemo and about half of these patients may have a remission. Stem cell transplant from a donor rarely works for blast phase CML. But it is more likely to work if the CML can be brought back to the chronic phase before the transplant. Treatment to relieve symptoms (called palliative treatment) is an important part of care for patients in this phase. Radiation treatment can help bone pain, and chemo may relieve some symptoms for a few months. In many cases, the effects of CML that has spread to the nervous system can be relieved by chemo or by radiation to the brain. Clinical trials are another option.
Clinical trials are one way to get state-of-the art cancer treatment. They are the only way for doctors to learn better methods to treat cancer. Still, they are not right for everyone. You can get a lot more information on clinical trials, in our document called Clinical Trials: What You Need to Know. You can read it on our Web site or call our toll-free number and have it sent to you.
Look for "red flags" that suggest fraud. Does the method promise to cure all or most cancers? Are you told not to have regular medical treatments? Is the treatment a "secret" that requires you to visit certain providers or travel to another country? Talk to your doctor or nurse about any method you are thinking of using. Contact us at 1-800-227-2345 to learn more about complementary and alternative methods in general and to find out about the specific methods you are looking at.
What are some questions I can ask my doctor about chronic myeloid leukemia?
As you cope with cancer and cancer treatment, you need to have honest, open talks with your doctor. Feel free to ask any question that's on your mind, no matter how small it might seem. Here are some questions you might want to ask. Be sure to add your own questions as you think of them. Nurses, social workers, and other members of the treatment team may also be able to answer many of your questions. What phase is my chronic myeloid leukemia (CML) in? What treatment choices do I have? Which treatment do you recommend, and why? What side effects are there to the treatments you recommend? What can I do to be ready for treatment? How often will you test my blood or bone marrow to see how my treatment is working? Should we think about a stem cell transplant at this time? What are the chances that the leukemia will come back once I am in remission? Add your own questions below:
Follow-up care
Even if there are no signs of the disease, your doctors will still want to watch you closely. It is very important to go to all of your follow-up doctor visits. During these, your doctors will ask questions about any problems you may have and may do exams and lab tests to look for signs of CML and treatment side effects. Almost any cancer treatment can have side effects. Some may last for a few weeks to months, but others can last the rest of your life. This is the time for you to talk to your cancer care team about any changes or problems you notice and any questions or concerns you have. It is important to keep health insurance. Tests and doctor visits cost a lot, and even though no one wants to think of their cancer coming back, this could happen. Should your cancer come back, our document, When Your Cancer Comes Back: Cancer Recurrence can give you information on how to manage and cope with this phase of your treatment.
If you were in the hospital, a copy of the discharge summary that the doctor wrote when you were sent home from the hospital If you had radiation treatment, a summary of the type and dose of radiation and when and where it was given If you had chemo or targeted therapies, a list of your drugs, drug doses, and when you took them
Eating better
Eating right can be hard for anyone, but it can get even tougher during and after cancer treatment. Treatment may change your sense of taste. Nausea can be a problem. You may not feel like eating and lose weight when you don't want to. Or you may have gained weight that you can't seem to lose. All of these things can be a challenge. If treatment caused weight changes or eating or taste problems, do the best you can and keep in mind that these problems most often get better over time. You may find it helps to eat small portions every 2 to 3 hours until you feel better. You may also want to ask your cancer team about seeing a dietitian an expert who can give you ideas on how to deal with these treatment side effects. One of the best things you can do after cancer treatment is put healthy eating habits into place. You may be surprised at the long-term benefits of some simple changes, like eating a greater variety of healthy foods. Getting to and staying at a healthy weight, eating a
healthy diet, and limiting your alcohol intake may lower your risk for a number of types of cancer, as well as having many other health benefits.
How does having chronic myeloid leukemia affect your emotional health?
Once your treatment ends, you may be surprised by the flood of emotions you go through. This happens to a lot of people. You may find that you think about the effect of your cancer on things like your family, friends, and career. Money may be a concern as the medical bills pile up. Or you may begin to think about the changes that cancer has brought to your relationship with your spouse or partner. Unexpected issues may also cause concern for instance, as you get better and need fewer doctor visits, you will see your health care team less often. This can be hard for some people.
This is a good time to look for emotional and social support. You need people you can turn to. Support can come in many forms: family, friends, cancer support groups, church or spiritual groups, online support communities, or private counselors. The cancer journey can feel very lonely. You don't need to go it alone. Your friends and family may feel shut out if you decide not include them. Let them in and let in anyone else who you feel may help. If you aren't sure who can help, call your American Cancer Society at 1-800-227-2345 and we can put you in touch with a group or resource that may work for you. You can't change the fact that you have had cancer. What you can change is how you live the rest of your life making healthy choices and helping your body and mind feel well.
Genetics of CML
Scientists are making progress in learning how changes in a person's DNA can cause normal bone marrow cells to change into leukemia. They are learning why these cancer cells grow too fast, live too long, and don't grow into normal blood cells. In recent years they have learned a lot about why cancer cells don't die off like normal cells. This information is being used to develop many new drugs.
Cancer vaccines
Because leukemia cells are a kind of foreign cell, it is possible to get the body to take action against them. Researchers are looking at ways to do this with cancer vaccines a substance injected into the body that boosts the immune system and causes it to attack certain cells. Some vaccines are now being studied for use against CML. More research into how to make and use vaccines is being done, too.
Leukemia & Lymphoma Society Toll-free number: 1-800-955-4572 Internet address: www.lls.org Leukemia Links Web site: www.acor.org/leukemia National Bone Marrow Transplant Link (nbmtLink) Toll-free number: 1-800-LINK-BMT (1-800-546-5268) Web site: www.nbmtlink.org National Cancer Institute Toll-free number1-800-4-CANCER (1-800-422-6237) Web site: www.cancer.gov National Marrow Donor Program Toll-free number: 1-800-MARROW2 (1-800-627-7692) Web site: www.marrow.org
*Inclusion on this list does not imply endorsement by the American Cancer Society.
No matter who you are we can help, contact us any time, day or night, for information and support. Call us at 1-800-227-2345 or visit www.cancer.org.
Last Medical Review: 6/14/2012 Last Revised: 6/14/2012 2012 Copyright American Cancer Society