Aids-Related Non-Hodgkin Lymphomas
AIDS-related non-Hodgkin lymphomas (AIDS-NHL) are almost invariably derived from B cells and are grouped into three distinct histologic categories, including small non cleaved cell lymphoma (SNCCL), diffuse large cell lymphoma (DLCL), and anaplastic large cell lymphoma (ALCL). In addition, AIDS-NHL presenting solely as a body cavity effusion are thought to be a peculiar clinico-pathologic entity and are defined as body-cavity-based lymphoma (BCBL). At the biologic level, AIDS-related lymphomagenesis is characterized by activation of proto-oncogenes, inactivation of tumor suppressor genes, viral infection of the tumor clone, and deregulated cytokine production. Distinct AIDS-NHL types associate with specific molecular pathways. The first pathogenetic pathway clusters with AIDS-SNCCL, and is characterized by a relatively mild degree of host immunodeficiency. AIDS-SNCCL consistently associates with c-MYC rearrangements and p53 inactivation in 100% and 60% of the cases respectively, whereas infection by Epstein-Barr virus (EBV) is restricted to 30% of the cases. Production of high levels of IL-10 is an additional peculiar feature of EBV positive AIDS-SNCCL. The second pathogenetic pathway associates with AIDS-DLCL, which is usually accompanied by a marked immunodeficiency of the host. AIDS-DLCL is characterized by EBV infection in the large majority of cases and by the mutually exclusive presence of BCL-6 rearrangements and c-MYC translocation in 40% of the cases. A third pathway characterizes AIDS-BCBL, which associates virtually in all cases with infection by EBV and with the presence of DNA sequence of the recently identified Kaposi sarcoma herpes virus (KSHV) in the apparent absence of other known genetic lesions. Finally, the pathogenetic features of AIDS-ALCL are still under investigation.
KeywordsAnaplastic Large Cell Lymphoma Genetic Lesion Diffuse Large Cell Lymphoma Chronic Antigen Stimulation Persistent Generalize Lymphadenopathy
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