Blood. 2020 Nov 10:blood.2019004099. doi: 10.1182/blood.2019004099. Online ahead of print.
Burkitt lymphoma (BL) is a highly aggressive, B-cell, non-Hodgkin lymphoma (NHL) categorized into endemic, sporadic and immunodeficiency-associated subtypes. BL has distinct pathologic and clinical features, characterized by rapidly progressive tumors with high rates of extranodal involvement. Next generation sequencing (NGS) analyses have further characterized the genomic landscape of BL and our understanding of disease pathogenesis, though these findings have yet to influence treatment.
Although the majority of patients are cured with intensive combination chemotherapy, given the paucity of randomized trials, optimal therapy has not been defined. Furthermore, treatment for elderly patients, patients with central nervous system (CNS) involvement, or those with relapsed disease, remains an unmet need. In this review, we highlight the clinical, pathologic, and genomic features, as well as standard and emerging treatment options for adult patients with BL.