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Malignant lymphoma, small non cleaved, Burkitt type [OBS]
Malignant lymphoma, undifferentiated, Burkitt type [OBS]
Highly aggressive NHL often presenting in extranodal sites such as the GI tract; the hallmark is over-expression of oncogene c-Myc.
There are three variants of Burkitt lymphoma (BL): Endemic BL, Sporadic BL and Immunodeficiency-associated BL. All of these variants are coded 9687/3. Each of these variants manifests differences in clinical presentation, morphology, and biology. In each of the clinical variants, patients are at high risk for central nervous system involvement.
Three clinical variants: 1. Endemic BL occurs in equatorial Africa and is the most common childhood malignancy in this area. Endemic BL is also endemic in Papua, New Guinea. The jaws and other facial bones (orbit) are the site of presentation in about 50% of cases. The distal ileum, cecum, anterior and/or omentum, gonads, kidneys, long bones, thyroid, salivary glands, and tonsils may be affected with or without jaw involvement. Localization may be found in BM, but manifestation of leukemia in peripheral blood is not present.
2. Sporadic BL is seen throughout the world, mainly in children. This variant is primarily seen in association with HIV infection often occurring as the initial manifestation of the acquired immunodeficiency syndrome (AIDS). Jaw tumors are very rare. Majority of cases present with abdominal masses. Retroperitoneal masses may result in spinal cord compression.
3. Immunodeficiency-associated-BL is seen in association with AIDS. It is often the first manifestation of AIDS. Nodal localization is frequent as well as bone marrow involvement.
Burkitt leukemia is the leukemic phase observed in patients with bulky disease, but only rare cases present purely as acute leukemia with peripheral blood and bone marrow involvement. (See 9826/3 for Burkitt leukemia)