Disease Information
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 Children’s 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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