Burkitt lymphoma

Burkitt lymphoma is a cancer of the lymphatic system, particularly B lymphocytes found in the germinal center. It is named after Denis Parsons Burkitt, the Irish surgeon who first described the disease in 1958 while working in equatorial Africa. The overall cure rate for Burkitt lymphoma in developed countries is about 90%, and it is worse in low-income countries. Burkitt lymphoma is uncommon in adults, in whom it has a worse prognosis. Burkitt lymphoma can be divided into three main clinical variants: the endemic, the sporadic, and the immunodeficiency-associated variants. By morphology (i.e., microscopic appearance), immunophenotype, and genetics, the variants of Burkitt lymphoma are alike. The endemic variant (also called "African variant") most commonly occurs in children living in regions where malaria is endemic (such as equatorial Africa, Brazil, and Papua New Guinea). Epstein–Barr virus (EBV) infection is found in nearly all patients. Chronic malaria is believed to reduce resistance to EBV, allowing EBV infection to occur. The disease characteristically involves the jaw or other facial bone, abdomen, cecum, distal ileum ovaries, kidney, or breast. Less than 10% of cases present with central nervous system involvement, which usually manifest as cranial nerve palsies or spinal cord compression. The sporadic type of Burkitt lymphoma (also known as "non-African") is the most common variant found in places where malaria is not endemic such as North America and parts of Europe. The median onset is 10 years of age, but there are also peaks at ages 40 and 75 years old. Males are 3-4 times more likely to be affected as compared to females. The tumor cells have a similar appearance to the those of the classical endemic Burkitt lymphoma. Sporadic type Burkitt lymphomas are less commonly associated with the EBV virus as compared to the endemic variant; with 20-30% of cases being attributed to EBV (most commonly in adults older than 50). The jaw is less commonly involved in this variant.