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 into 3 clinical variants: endemic Burkitt's lymphoma, sporadic Burkitt's lymphoma, and immunodeficiency-associated Burkitt's lymphoma.

Classification

Burkitt's lymphoma may be classified 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.

Burkitt's lymphoma classification

Name	Description
Endemic Burkitt's lymphoma	Also called African Burkitt's lymphoma because it mainly occurs in Africa 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	Also called 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-Hodgkins, 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 of immunodeficiency-associated Burkitt's lymphoma appears to be dropping because of antiretroviral therapy (a combination of anti-HIV drugs used for treating HIV infection) About 30–40% of cases are associated with the Epstein-Barr virus

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