RT Book, Section A1 Evans, Andrew G. A1 Friedberg, Jonathan W. A2 Kaushansky, Kenneth A2 Lichtman, Marshall A. A2 Prchal, Josef T. A2 Levi, Marcel M. A2 Press, Oliver W. A2 Burns, Linda J. A2 Caligiuri, Michael SR Print(0) ID 1121100683 T1 Burkitt Lymphoma T2 Williams Hematology, 9e YR 2015 FD 2015 PB McGraw-Hill Education PP New York, NY SN 9780071833004 LK hemonc.mhmedical.com/content.aspx?aid=1121100683 RD 2024/10/13 AB SUMMARYBurkitt lymphoma is one of the highly aggressive B-cell lymphomas. It was the first tumor to be etiologically associated with (1) a virus, specifically Epstein-Barr virus, (2) a specific translocation involving the MYC oncogene, and (3) one of the first cancers shown to be curable by chemotherapy alone. It presents in three clinically distinct forms: endemic, sporadic, and immunodeficiency-associated. Burkitt lymphoma is an uncommon form of lymphoma in adults, with an incidence of approximately 1200 patients per year in the United States. Over the last decade, the definition of Burkitt lymphoma has been refined, largely as a consequence of improvements in immunohistochemical, cytogenetic, and molecular diagnostic techniques. Transcriptional profiling has more clearly defined Burkitt lymphoma at the molecular level, while whole-genome sequencing has expanded our understanding of the mutational landscape that underlies this disease. Despite these refinements in diagnostic criteria, the differential diagnosis includes several high-grade lymphomas, including a group of patients with a diagnosis defined by the World Health Organization as intermediate between Burkitt lymphoma and diffuse large B-cell lymphoma. Burkitt lymphoma is a highly curable malignancy in the modern therapeutic era. The majority of younger patients are cured with intensive chemotherapeutic regimens, and increasing efficacy has been demonstrated in older patients with reduced intensity treatments. Remaining challenges include the optimal management of older patients, the development of therapy for patients with relapsed or refractory disease, and the translation of gains made in treatment to the management of endemic disease.