Also called Burkkit cell leukemia, it is an uncommon type of monoclonal B-cell non-Hodgkin lymphoma (Johnston et. al, 2012) that is rare in the United States, but is endemic in equatorial Africa, Brazil, and Papua New Guinea. This rare disease is associated with Epstein-Barr virus and “was one of the first tumors shown to have a chromosomal translocation that activates an oncogene (c-MYC)” (Molyneux et. al, 2012). It is the fastest growing human tumor. It is more common in men (both children and adults) and in patients with HIV / AIDS.
In most cases, it appears as a voluminous abdominal disease, which often originates in the region of the ileocecal valve. In adults, it can be voluminous and generalized, often with massive involvement of the liver, spleen and bone marrow, and the pathological anatomy demonstrates a high mitotic index and a pattern of rapidly proliferating malignant lymphocytes. Brain and cerebrospinal fluid involvement are often seen at the time of diagnosis. The diagnosis is confirmed with a bone marrow biopsy and the treatment goes from chemotherapy to bone marrow transplantation.
Johnston, W. Thomas, et al. "Relationship between Plasmodium falciparum malaria prevalence, genetic diversity and endemic Burkitt lymphoma in Malawi." Scientific reports 4 (2014): 3741.
Molyneux, Elizabeth M., et al. "Burkitt's lymphoma." The Lancet 379.9822 (2012): 1234-1244.