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Hodgkin and non-Hodgkins lymphomas constitute 1015% of all childhood cancers in the more developed countries slight overall

male predominance in the incidence of HL, which is most marked in children below 10 years of age, while in adolescents, the incidence between males and females is roughly equal at 0.5 HL is rarely diagnosed in children under 5

Leukemias / Lymphomas: Non-Hodgkin Lymphoma (NHL)


Alternate Names: NHL

Definition
Non-Hodgkin lymphomas (NHL) are tumors of the peripheral lymph nodes, thymus or abdominal organs, such as the bowel, but can appear in other sites. These tumors differ substantially from lymphomas seen in adults. In children, NHL may behave similarly to acute leukemia in that both cancers can involve bone marrow, blood, skin and the central nervous system.

Incidence
Lymphomas are the third most common malignant disease of children, after acute leukemia and brain tumors. Fifty-five percent of childhood lymphomas are non-Hodgkin lymphomas. Approximately nine in one million children under age 15 will develop non-Hodgkin lymphoma each year.

Influencing Factors
Children with immunodeficiencies have a greatly increased risk of developing non-Hodgkin lymphoma.

Survival Rates
Approximately 80 percent of patients with non-Hodgkin lymphoma can be cured with treatment. Children with early, localized stages of the disease have about a 90 percent chance of cure.

Treatment Strategies

Therapy varies depending on subtypes and stages of non-Hodgkin lymphoma. Chemotherapy, using a combination of drugs, has proven effective. Because of the effectiveness of chemotherapy, there is little role for radiation treatment.

Current Research
Researchers are looking for better ways to refine treatment for lymphoma according to the extent of the disease and the tissue subtype. Specific therapies are being developed to target against cell surface antigens expressed by lymphoma cells or their molecular lesions. Molecular studies are being conducted to search for the mechanisms of the development of lymphoma

Leukemias / Lymphomas: Hodgkin Lymphoma


Alternate Names: Hodgkin disease

Definition
Hodgkin lymphoma is a cancer characterized by progressive enlargement of affected lymph nodes and sometimes spread to the spleen, liver, bone marrow, bones, or lungs.

Incidence

Every year in the United States, 6,000 to 7,000 new cases of Hodgkin lymphoma are diagnosed (in adults and children).. In the United States and northern Europe, Hodgkin lymphoma is rare before the age of five, with a gradual rise in incidence until adolescence and a peak in the 20s and another peak later in adulthood.

Influencing Factors

Boys are more likely than girls to develop Hodgkin lymphoma. Siblings of patients have a slightly increased risk of developing the disease. The virus that causes mononucleosis (Epstein-Barr virus) is believed to play a role in the development of some cases of Hodgkin lymphoma.

Survival Rates

The survival rate today for low-stage Hogkin lymphoma is greater than 90 percent. The survival rate for advanced disease is greater than 80 percent.

Treatment Strategies

Radiation therapy works well for localized Hodgkin lymphoma, but may significantly impair the growth of bones and soft tissues in young children and increase the risk of heart disease and second cancers as patients grow older. Chemotherapy alone or combination therapy with chemotherapy and lowdose radiation therapy is the standard treatment for young patients and those who have more advanced disease.

Current Research

Newer risk-adapted therapies are under investigation to determine if it is possible to reduce the amount of treatment (which should reduce the longterm side effects of heart and lung disease and second cancers) and still maintain cure rates. Three treatment protocols are currently available at St. Jude for patients with Hodgkin lymphoma: HOD99, HOD05 and HOD08. The Hodgkin consortium is based at St Jude and led by Monika Metzger, MD. It includes the following institutions: Dana Farber Cancer Institute, Stanford Comprehensive Cancer Center, Massachusetts General Hospital Cancer Center and Maine Medical Center. Collaborators work together to develop improved treatment strategies for Hodkgin lymphoma patients. Long term side effects in survivors of Hodgkin lymphoma are being investigated in the SJLIFE studies 4,5 and 16. Laboratory-based scientists are working with physicians to better understand the role that viruses like the Epstein-Barr virus play in the development of Hodgkin lymphoma. This information will be helpful in developing new approaches to treat Hodgkin lymphoma.

Leukemias / Lymphomas: Acute Lymphoblastic Leukemia (ALL)


Alternate Names: ALL, acute lymphocytic leukemia, acute lymphoid leukemia

Definition

Acute lymphoblastic leukemia (ALL) is the most common form of childhood cancer. It affects lymphocytes, a type of white blood cells. Leukemic cells accumulate in the bone marrow, replace normal blood cells and spread to other organs including liver, spleen, lymph nodes, central nervous system, kidneys and gonads.

Incidence
In the United States, about 3,000 children each year are found to have acute lymphoblastic leukemia. Peak incidence occurs from 3 to 5 years of age.

Influencing Factors
ALL affects slightly more boys than girls. It occurs more frequently among whites than blacks. Although siblings of leukemic children have a slightly higher risk of developing the disease, the incidence is relatively low (no more than one in 500).

Survival Rates
About 98 to 99 percent of children with newly diagnosed acute lymphoblastic leukemia attain initial complete remissions (absence of detectable leukemic cells by microscopic examination) in four to six weeks. About 90 percent of children can be cured. Patients who remain leukemia-free for 10 years or more can be considered cured.

Treatment Strategies
Chemotherapy is used to kill leukemia cells. All chemotherapy is stopped after two to three years of treatment. Radiation therapy, which used to be a standard component of leukemia treatment, can now safely be eliminated for the majority of children with ALL. Hematopoietic stem cell transplantation is an option for very high-risk cases (e.g., Philadelphia chromosome-positive ALL or slow responders to remission induction therapy) or those who develop an early relapse in the bone marrow.

Current Research

Research aims to improve treatment outcome by optimizing dosage and scheduling of antileukemic agents based on the individual patients leukemic cell genetic features and host normal cell pharmacogenetics characteristics. Molecular genetic abnormalities and the measurement of the level of minimal residual leukemia after remission induction therapy are used to

help direct the type of treatment. Research on the profile of gene and protein expression in leukemia cells using microarray technology is providing new insights to the genomics of leukemia and the effects of treatment; this research may lead to unique molecular targets for developing new therapies that are more specific and effective. Pharmacogenomic studies are providing new insights to inherited differences in drug response and to individualize treatment. Methods are under study to improve the efficacy and safety of stem cell transplantation and to increase the number of potential of donors. Use of novel agents in children at a high risk of relapse. Development of effective treatment regimens for relapsed ALL.

Leukemias / Lymphomas: Acute Myeloid Leukemia (AML)


Alternate Names: AML, non-lymphoid, myeloblastic, granulocytic, myelocytic leukemia

Definition
Acute myeloid leukemia (AML ) affects various white blood cells including granulocytes, monocytes and platelets. Leukemic cells accumulate in the bone marrow, replace normal blood cells and can spread to the liver, spleen, skin, or central nervous system.

Incidence

Approximately 500 children are diagnosed with acute myeloid leukemia in the United States each year. AML is diagnosed in about 20 percent of children with leukemia. AML is the most common second malignancy (a different or second cancer found in a patient previously treated for cancer) in children treated for malignancies.

Influencing Factors
There is a greater incidence of leukemia among people exposed to large amounts of radiation and certain chemicals (e.g. benzene).

Survival Rates
Although approximately 80 to 90 percent of children with acute myeloid leukemia attain remissions (absence of leukemic cells), some of those patients have later recurrences. About 70 percent of children with AML achieve long-term remissions with chemotherapy or stem cell transplantation.

Treatment Strategies

Chemotherapy is the most common form of therapy for children with AML. Allogeneic stem cell (harvested from bone marrow, cord blood or peripheral blood) transplantation is preferred treatment for those patients with AML who are at a high risk of relapse or who have disease that is resistant to other treatments. Allogeneic transplants use stem cells from a donor.

Current Research
St. Jude Children's Research Hospital has committed substantial resources to study the fundamental mechanisms of acute myeloid leukemia. In addition, St. Jude investigators are trying innovative forms of treatment to improve the outcome of this deadly disease. Current clinical trials include:

intensive use of chemotherapy plus stem cell transplantation; clofarabine, a novel agent for the treatment of AML; natural killer cell transplantation; treatment that is based on the specific subtype of AML and on the response to therapy; the monitoring of minimal residual disease by flow-cytometric and molecular techniques; investigation of genetic abnormalities in AML using novel methods.

Leukemias / Lymphomas: Acute Promyelocytic Leukemia (APL)


Alternate Names: Acute myeloid leukemia M3 under French-American-British classification

Definition
Acute promyelocytic leukemia (APL) is a subtype of the cancer acute myeloid leukemia (AML). APL is characterized by abnormal, heavily granulated promyelocytes, a form of white blood cells. APL results in the accumulation of these atypical promyelocytes in the bone marrow and peripheral blood, and replaces normal blood cells.

Incidence

Approximately 50 children are diagnosed with APL, out of the estimated 500 children diagnosed each year with AML in the United States. APL represents about one percent of childhood leukemia. APL is found more often in children between the ages of two and three, and in adults over 40, however it has also been found in older children and

teenagers. APL is found more frequently in children of Hispanic and Mediterranean origin.

Survival Rates
With the combination treatment of chemotherapy and ATRA about 75-80 percent of young patients are expected to be long term survivors.

Treatment Strategies

Chemotherapy is used in combination with all trans retinoic acid (ATRA). Some centers, including St. Jude, are testing forms of arsenic trioxide for treating APL.

Current Research
Open protocol at St. Jude:

AML0631: Risk adapted treatment of newly diagnosed childhood APL using arsenic trioxide during consolidation. This multi-institutional Phase III study is an effort of the Childrens Oncology Group to treat newly diagnosed children with APL. The goal of this study is to reduce the dose of chemotherapeutic drugs that are toxic to the heart while continuing to provide an excellent cure rate. Arsenic trioxide which is a very effective agent in APL will be used in this study. In addition, biologic tests will be done to develop improve understanding of this disease.

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