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INTRODUCTION

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SUMMARY

Burkitt 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.

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Acronyms and Abbreviations

B-ALL, acute B-cell lymphoblastic leukemia; BCL-U, B-cell lymphoma–unclassifiable; BL, Burkitt lymphoma; B-LBL, B-cell lymphoblastic lymphoma; CODOX-M/IVAC, cyclophosphamide, doxorubicin, vincristine, methotrexate, ifosfamide, etoposide and high-dose cytarabine, with intrathecal cytarabine and methotrexate; DHL, double-hit lymphoma; DLBCL, diffuse large B-cell lymphoma; EBER, Epstein-Barr virus-encoded RNA; eBL, endemic Burkitt lymphoma; EBNA, Epstein-Barr nuclear antigen; EBV, Epstein-Barr virus; FISH, fluorescence in situ hybridization; GEP, gene-expression profiling; HAART, highly active antiretroviral therapy; hyper-CVAD, fractionated cyclophosphamide, vincristine, doxorubicin, dexamethasone; Ig, immunoglobulin; LDH, lactate dehydrogenase; NHL, non-Hodgkin lymphoma; PTLD, posttransplantation lymphoproliferative disease; R-EPOCH, etoposide, vincristine and doxorubicin, with bolus rituximab, cyclophosphamide and steroids; WHO, World Health Organization.

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DEFINITION AND HISTORY

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Burkitt lymphoma (BL) may present in three distinct forms: endemic (African), sporadic, and immunodeficiency-associated.1 The endemic form (eBL) is the most common pediatric tumor in sub-Saharan Africa and other regions of the world where malaria is endemic. It typically presents in the jaw or maxilla, and is associated with Epstein-Barr virus (EBV) infection at an early age. Although there are reports dating to as early as 1910, it was Denis Burkitt who is credited with describing this malignancy in 1958 as a common tumor in children of Uganda.2,3 Originally thought by Burkitt to be a sarcoma of the jaw, it was a pathologist named George O’Connor who, in 1960, concluded it was a lymphoma.4 In 1964, while studying BL samples by electron microscopy, Sir Michael Anthony Epstein, Yvonne Barr, and ...

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