Burkitt lymphoma is reported by the World Health Organization as a highly aggressive B-cell lymphoma. Three clinical variants of Burkitt lymphoma are recognized (endemic, sporadic, immunodeficiency-associated), which differ in geographic distribution, clinical presentation, and association with infectious agents and cell biology. The tumor is composed by monomorphic medium-sized B-cells with basophilic cytoplasm, numerous mitotic figures, and an extremely high proliferation rate (Ki-67-index >95 %). A “starry sky” pattern is usually present, due to numerous benign macrophages that have ingested apoptotic tumor cells. Tumor cells express membrane IgM with light chain restriction and B-cell-associated antigens CD19, CD20, CD22, and CD79a. Chromosomal translocation involving MYC is the most frequent genetic feature; however, some cases lack MYC translocation. No singleparameter (such as morphology, genetic analysis, or immunophenotyping) can be used as the gold standard for the diagnosis of BL, but a combination of several diagnostic techniques is necessary.
Leoncini, L., Stein, H. (2013). Definition of Burkitt Lymphoma. In Erle S. Robertson (a cura di), Burkitt’s Lymphoma (pp. 81-93). New York : Springer [10.1007/978-1-4614-4313-1_5].
Definition of Burkitt Lymphoma
Leoncini L.;
2013-01-01
Abstract
Burkitt lymphoma is reported by the World Health Organization as a highly aggressive B-cell lymphoma. Three clinical variants of Burkitt lymphoma are recognized (endemic, sporadic, immunodeficiency-associated), which differ in geographic distribution, clinical presentation, and association with infectious agents and cell biology. The tumor is composed by monomorphic medium-sized B-cells with basophilic cytoplasm, numerous mitotic figures, and an extremely high proliferation rate (Ki-67-index >95 %). A “starry sky” pattern is usually present, due to numerous benign macrophages that have ingested apoptotic tumor cells. Tumor cells express membrane IgM with light chain restriction and B-cell-associated antigens CD19, CD20, CD22, and CD79a. Chromosomal translocation involving MYC is the most frequent genetic feature; however, some cases lack MYC translocation. No singleparameter (such as morphology, genetic analysis, or immunophenotyping) can be used as the gold standard for the diagnosis of BL, but a combination of several diagnostic techniques is necessary.
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https://hdl.handle.net/11365/1058143