Burkitt's lymphoma classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]

Overview

Burkitt's lymphoma may be classified according to WHO classification into 3 clinical variants: endemic Burkitt's lymphoma, sporadic Burkitt's lymphoma, and immunodeficiency-associated Burkitt's lymphoma.

Classification

WHO Classification based on the geographic distribution and clinical presentation

Burkitt's lymphoma may be classified according to WHO classification into 3 clinical variants:

• Endemic Burkitt's lymphoma
• Sporadic Burkitt's lymphoma
• Immunodeficiency-associated Burkitt's lymphoma

By morphology (i.e. microscopic appearance) or immunophenotype, it is almost impossible to differentiate these three clinical variants. Immunodeficiency-associated Burkitt's lymphoma may demonstrate more plasmacytic appearance or more pleomorphism, but these features are not specific.

WHO classification based on the geographic distribution and clinical presentation

Name	Description
Endemic Burkitt's lymphoma (African Burkitt's lymphoma)	The most common childhood cancer in Africa Almost always associated with Epstein-Barr virus infection Characteristically involves the jaw or other facial bone, distal ileum, cecum, ovaries, kidney, or the breast
Sporadic Burkitt's lymphoma (Non-African or Non-endemic Burkitt's lymphoma)	The most common type of Burkitt's lymphoma in North America and European countries Sometimes associated with the Epstein-Barr virus, but in many cases this virus is not present Non-Hodgkin's lymphoma, which includes Burkitt's, accounts for 30-50% of childhood lymphoma Ileo-cecal region is the common site of involvement Most often starts in the abdomen, but can also start in the ovaries, testicles, kidneys or other organs Jaw is less commonly involved, comparing with the endemic variant Can also involve the pleura lining the lung, central nervous system (CNS) and, uncommonly, the breast
Immunodeficiency-associated Burkitt's lymphoma	Tends to occur in people with weakened immune systems, typically those with HIV infection Incidence appears to be dropping because of antiretroviral therapy About 30–40% of cases are associated with the Epstein-Barr virus

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