Adult Burkitt Leukemia/Lymphoma

Burkitt lymphoma (BL) and Burkitt cell acute lymphoblastic leukemia (L3ALL) are different clinical presentations of a single highly aggressive B-cell non-Hodgkin lymphoma initially described by Dennis Burkitt in 1958. The three clinical forms of this disease, i.e., endemic, sporadic, and immunodeficiency-related, result from the constitutive expression of MYC proto oncogene located on chromosome 8q24. BL presents as a rapidly growing tumor, with a very short doubling time and a quick dissemination to extra nodal sites including the bone marrow and the central nervous system (CNS). Tumor lysis syndrome is a serious and potentially fatal complication that occurs spontaneously or upon initiation of chemotherapy and often requires admission into the intensive care unit and initiation of hemodialysis. Patient outcomes improved with the adoption of pediatric-inspired short-course dose-intensive chemotherapy regimens, usually preceded by a prephase induction using low doses of steroids and chemotherapy to prevent massive tumor lysis syndrome. Addition of rituximab to chemotherapy has become a standard of care and aggressive CNS-oriented therapy. Relapsed/refractory disease has a very poor prognosis, and the benefit from autologous/allogeneic hematopoietic stem cell transplant remains uncertain. Despite the significant improvement in complete remission and survival rates, treatment of BL remains problematic in specific patient groups such as the elderly or immunocompromised patients. Novel and less toxic therapies are underway.