This is also more common in children. It is associated with translocations involving the c-myc gene on chromosome 8 rearranging with immunoglobulin heavy or light chain genes. Pts often have disseminated disease with large abdominal masses, hepatomegaly, and adenopathy. If a leukemic picture predominates, it is classified as FAB L3.
Treatment: Burkitt's Lymphoma/Leukemia Resection of large abdominal masses improves treatment outcome. Aggressive leukemia regimens that include vincristine, cyclophosphamide, 6-mercaptopurine, doxorubicin, and prednisone are active. CODOX-M and the BFM regimen are the most effective regimens. Cure may be achieved in 50-60%. The need for maintenance therapy is unclear. Prophylaxis against tumor lysis syndrome is important (Chap. 25. Oncologic Emergencies). |
Section 6. Hematology and Oncology