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Burkitt lymphoma in Adult
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Burkitt lymphoma in Adult

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Contributors: Amanda Truong BS, Jason E. Hawkes MD, Lowell A. Goldsmith MD, MPH
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Synopsis

Burkitt lymphoma (BL) is an aggressive form of non-Hodgkin B-cell lymphoma typically presenting in the first and second decades of life. This tumor very commonly is associated with a translocation of chromosome 8 and 14, causing overexpression of the c-Myc proto-oncogene. Tumors may present in the abdomen, kidney, testes, breast, central nervous system (CNS), bone marrow, and head / neck region. There are 3 major variants: endemic, sporadic, and immunodeficiency (often HIV) associated BL.

The endemic form is strongly associated with the Epstein-Barr virus (EBV) and is twice as common in males. Approximately half of endemic BL cases present with a jaw or facial bone tumor. Coinfection with malaria greatly increases the risk of endemic BL, and, therefore, this variant is commonly seen in areas where malaria is endemic, such as Africa, Brazil, and New Guinea.

The sporadic form is commonly seen in the United States and Western Europe. This variant is approximately 3-4 times more common in males. The typical presentation is an abdominal mass, with approximately one-quarter of cases presenting with a jaw or facial tumor.

The immunodeficiency-associated form of BL is associated with AIDS or other immunodeficient states (eg, organ transplant recipients). HIV patients who develop BL typically have a CD4 count greater than 200. The risk of immunodeficiency-associated BL in HIV patients does not decrease with highly active antiretroviral therapy (HAART). Lymph node, bone marrow, and CNS involvement are common for this BL variant and may lead to life-threatening tumor lysis syndrome.

Cutaneous manifestations of BL are exceeding rare. The pathogenesis of BL-associated skin lesions is unclear, but has been suggested to be the result of direct extension, hematogenous spread, or iatrogenic tumor seeding during procedures or trauma.

For more information, see OMIM.

Related topic: Non-Hodgkin lymphoma

Codes

ICD10CM:
C83.7 – Burkitt lymphoma, unspecified site

SNOMEDCT:
118617000 – Burkitt's Lymphoma

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Last Updated: 10/19/2017
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Burkitt lymphoma in Adult
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Burkitt lymphoma (Endemic) : Cervical lymphadenopathy, Fever, Night sweats, Edema, Hyperuricemia, LDH elevated, Weight loss, Anemia
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