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Burkitt lymphoma is one of the highly aggressive lymphomas. It was the first tumor to be etiologically associated with (1) a virus, specifically Epstein-Barr virus, (2) a specific chromosomal translocation involving chromosome 8, 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. It represents a highly curable malignancy in the modern therapeutic era. Over the last decade, the definition of Burkitt lymphoma has evolved significantly, largely as a consequence of improvements in immunohistochemical, cytogenetic, and molecular diagnostic techniques, and an increased understanding of the molecular basis of this disease. Modern techniques have more clearly defined Burkitt lymphoma at the molecular level. A group of patients have an ambiguous diagnosis between Burkitt lymphoma and diffuse large B-cell lymphoma, now defined by the World Health Organization, many of whom have a very poor prognosis. With intensive chemotherapeutic regimens, the majority of patients with Burkitt lymphoma are cured. Future studies are needed to best define therapy in older patients, and in the setting of relapsed or refractory disease.

Acronyms and Abbreviations

Abbreviations and acronyms used in this chapter: AID, activation-induced cytosine deamination; BL, Burkitt lymphoma; CNS, central nervous system; CODOX-M/IVAC, cyclophosphamide, doxorubicin, vincristine, methotrexate, ifosfamide, etoposide and high-dose cytarabine, with intrathecal cytarabine and methotrexate; EBER, Epstein-Barr virus-encoded RNA; eBL, endemic Burkitt lymphoma; EBNA, Epstein-Barr nuclear antigen; EBV, Epstein-Barr virus; FISH, fluorescence in situ hybridization; GC, germinal center; HAART, highly active antiretroviral therapy; hyper-CVAD, fractionated cyclophosphamide, vincristine, doxorubicin, dexamethasone; NHL, non-Hodgkin lymphoma; WHO, World Health Organization.

Burkitt lymphoma (BL) may present in three distinct forms: endemic (African), sporadic, and immunodeficiency associated.1–4 The endemic form (eBL) 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 lymphoma as a common tumor in children of Uganda.5 Further studies by Burkitt and others led to associations with EBV and malaria,6 as well as environmental factors.7 Subsequently, tumors of a similar histopathologic appearance were identified in nonendemic regions, and these were found to occur in older individuals with only occasional association with EBV; presentation was typically abdominal rather than orofacial.8 A third class of BL was identified in immunosuppressed patients, typically HIV-positive. Evidence for involvement of the MYC gene came with the identification of recurrent translocations involving the long arm of chromosome 8 in BL,9 that all involved MYC.

The endemic form is found in eastern equatorial Africa, with a peak age incidence at 4 to 7 years, and is nearly twice as frequent in boys as in girls. It accounts for 20 percent ...

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