Burkitt's and Burkitt-like lymphoma

Abstract

Burkitt's and Burkitt-like lymphoma (BL/BLL) are aggressive B-cell malignancies with a high proliferative rate that may be fatal within months if not treated promptly. Furthermore, treatment of BL/BLL requires comprehensive supportive care to avoid disease-related complications such as acute renal failure secondary to tumor lysis syndrome. Improvements in our understanding of the biology of BL and BLL have led to more effective therapeutic protocols. Clinical trials have demonstrated that short duration, multi-agent, dose-intensive chemotherapy regimens combined with aggressive central nervous system therapy results in long-term survival rates in children and young adults near 70% to 80%, whereas long-term disease-free survival rates in older adults remains suboptimal at 15% to 25%. Outcomes in HIV-associated BL/BLL are improved because of more effective chemotherapy regimens and enhanced HIV care. Autologous bone marrow transplantation has proven feasible in many patient populations with BL/BLL and may lead to cure in selected patients. Improved therapeutic strategies are warranted, such as integrating agents such as monoclonal antibodies to combination dose-intensive chemotherapy. Moreover, further study into the molecular biology of BL/BLL with attention to the role of c-myc dysregulation is needed to help predict prognostic factors and for the development of molecular targeted therapies. Clinical trials remain critical to determine the most effective treatment regimens that will continue to improve cure rates in this aggressive but treatable disease.