Burkitt's lymphoma classification
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Classification
Currently Burkitt's lymphoma can be divided into three main clinical variants: the endemic, the sporadic and the immunodeficiency-associated variants. Endemic Burkitt lymphoma also called African Burkitt lymphoma because it mainly occurs in Africa the most common childhood cancer in Africa almost always associated with Epstein-Barr virus infection commonly affects the jaw or facial bones
Sporadic Burkitt lymphoma
also called non-African or non-endemic Burkitt lymphoma the most common type of BL in North America and European countries sometimes associated with the Epstein-Barr virus, but in many cases this virus is not present most often starts in the abdomen, but can also start in the ovaries, testicles, kidneys or other organs can also involve the pleura lining the lung, central nervous system (CNS) and, uncommonly, the breast
Immunodeficiency-associated Burkitt lymphoma
tends to occur in people with weakened immune systems, typically those with HIV infection incidence of immunodeficiency-associated BL 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
- The endemic variant occurs in equatorial Africa. It is the most common malignancy of children in this area. Children affected with the disease often also had chronic malaria which is believed to have reduced resistance to the Epstein-Barr virus and allowed it to take hold. Disease characteristically involves the jaw or other facial bone, distal ileum, cecum, ovaries, kidney or the breast.
- The sporadic type of Burkitt lymphoma (also known as "non-African") is another form of non-Hodgkin lymphoma found outside of Africa. The tumor cells have a similar appearance to the cancer cells of classical African or endemic Burkitt lymphoma. Again it is believed that impaired immunity provides an opening for development of the Epstein-Barr virus. Non-Hodgkins, which includes Burkitt's, accounts for 30-50% of childhood lymphoma. Jaw is less commonly involved, comparing with the endemic variant. Ileo-cecal region is the common site of involvement.
- Immunodeficiency-associated Burkitt lymphoma is usually associated with HIV infection or occurs in the setting of post-transplant patients who are taking immunosuppressive drugs. Actually, Burkitt lymphoma can be the initial manifestation ofAIDS.
By morphology (i.e. microscopic appearance) or immunophenotype, it is almost impossible to differentiate these three clinical variants. Immunodeficiency-associated Burkitt lymphoma may demonstrate more plasmacytic appearance or more pleomorphism, but these features are not specific.