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:[1]

  • 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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