Burkitt lymphoma

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Disease Overview

Burkitt lymphoma (BL) is a very fast-growing type of cancer. It is a form of B-cell non-Hodgkin’s lymphoma.[13846] There are 3 recognized forms of BL:

  • Endemic (African) – the most common form, found mainly in central Africa, where it is associated with the Epstein Barr virus (EBV).[5293] It is most common in children. This form often manifests as enlargement of the jaw or facial bones.[13847]
  • Sporadic – a rarer form, seen in all parts of the world, that often develops in the abdomen with bone marrow involvement. The kidneys, ovaries, breasts or other organs may also be involved.[13847][5293] This form commonly affects children and young adults.[13855]
  • Immunodeficiency-associated – occurs primarily in people with HIV infection, and less commonly in people with other immunodeficiency disorders or recipients of organ transplants.[5293][13848]

Signs and symptoms may differ depending on the form of BL and the organs or body systems involved.[5293] When it spreads, weakness and fatigue often develop. Lymphoma cells may build up in the lymph nodes and other organs, causing swelling.[13846] Central nervous system involvement is possible with all forms of BL, particularly when there is advanced-stage disease.[13855]

The exact cause of BL is not known. EBV infection appears to play a role in virtually all cases of endemic (African) BL, and a minority of sporadic and immunodeficiency-associated BL.[13848] While acquired (not inherited) genetic changes involving the MYC gene and other genes are present within BL cancer cells, it is unclear what causes these genetic changes to occur.[5293][13847]

Without timely treatment, BL is rapidly fatal. Treatment involves intensive chemotherapy, which includes chemotherapy to the fluid surrounding the brain and spinal cord.[13846] The majority of people treated with aggressive therapy achieve long-term remission.[13848]


Synonyms

  • Burkitt's lymphoma
  • BL

For more information, visit GARD.

National Organization for Rare Disorders