Treating Burkitt Lymphoma in Adults

Abstract

Burkitt lymphoma is an uncommon form of aggressive lymphoma affecting approximately 1200 patients per year in the USA. It is characterized by a translocation involving the MYC oncogene. Three subtypes of Burkitt lymphoma are recognized: the endemic form, occurring primarily in Africa and associated with the Epstein-Barr virus (EBV); the sporadic form, representing less than 3 % of all non-Hodgkin lymphomas (NHL); and the immunodeficiency-associated form, occurring primarily in HIV-infected patients. Burkitt lymphoma appears histologically with a diffuse pattern of intermediate-sized monomorphic B cells, multiple nucleoli, a very high proliferative rate, and frequent mitotic figures. Recent advances in transcriptional profiling have improved the current molecular understanding of Burkitt lymphoma and have better characterized its mutational landscape. Most Burkitt lymphoma patients are cured with intensive treatment; however, prognosis is poor in elderly patients and those with relapsed disease.