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Primary Effusion Lymphoma

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Abstract

Primary effusion lymphoma (PEL) is a rare non-Hodgkin’s lymphoma that characteristically presents as a malignant effusion in the body cavities of patients with AIDS and occasionally in other patients with an underlying immunodeficiency. The malignant PEL cells are infected with Kaposi’s sarcoma herpes virus (KSHV, also known as human herpesvirus-8, HHV8) and are often co-infected with Epstein–Barr virus (EBV). PEL cells display morphologic features of large-cell lymphomas and are of B-cell lineage. The diagnosis of PEL is characteristically made in AIDS patients presenting with a malignant effusion in the pleural, pericardial, or peritoneal cavity characterized by the presence of KSHV-infected cells with characteristic morphology and phenotype. The clinical course of PEL is generally aggressive, with a mean survival of less than 1 year, despite chemotherapy.

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Tosato, G. (2014). Primary Effusion Lymphoma. In: Yarchoan, R. (eds) Cancers in People with HIV and AIDS. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-0859-2_14

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