Definition
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-associated herpesvirus (KSHV, also known as human herpesvirus-8, HHV8) and are often coinfected 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.
Introduction
PEL was recognized as a distinct clinical entity when it was discovered that certain...
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Tosato, G. (2018). Primary Effusion Lymphoma. In: Hope, T.J., Richman, D.D., Stevenson, M. (eds) Encyclopedia of AIDS. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-7101-5_4
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