TY - CHAP M1 - Book, Section TI - Burkitt Lymphoma A1 - Evans, Andrew G. A1 - Friedberg, Jonathan W. A1 - Casulo, Carla A2 - Kaushansky, Kenneth A2 - Prchal, Josef T. A2 - Burns, Linda J. A2 - Lichtman, Marshall A. A2 - Levi, Marcel A2 - Linch, David C. Y1 - 2021 N1 - T2 - Williams Hematology, 10e AB - SUMMARYBurkitt lymphoma is one of the highly aggressive B-cell lymphomas. It was the first tumor to be etiologically associated with (a) a virus, specifically Epstein-Barr virus, (b) a specific translocation involving the MYC oncogene, and (c) one of the first cancers shown to be curable by chemotherapy alone. It presents in three clinically distinct forms: endemic, sporadic, and immunodeficiency associated. BL is an uncommon form of lymphoma in adults, with an incidence of approximately 1200 patients per year in the United States. Over the past decade, the definition of BL has been refined, largely as a consequence of improvements in immunohistochemical, cytogenetic, and molecular diagnostic techniques. Transcriptional profiling has more clearly defined BL at the molecular level, and 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 aggressive lymphomas, including a group of patients with a diagnosis defined by the World Health Organization (WHO) as high-grade B-cell lymphoma with MYC and BCL2 or BCL6 rearrangements and a new WHO provisional entity called Burkitt-like lymphoma with chromosome 11q aberration. BL 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 with reduced-intensity treatments in older patients. 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. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/29 UR - accessmedicine.mhmedical.com/content.aspx?aid=1180444416 ER -