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AIDS-related lymphomas: Primary effusion lymphoma

Lawrence D Kaplan, MD
Wei Ai, MD, PhD
Section Editor
Arnold S Freedman, MD
Deputy Editor
Alan G Rosmarin, MD


Infection with the human immunodeficiency virus (HIV) predisposes to the development of neoplasms, including lymphoma. Acquired immune deficiency syndrome (AIDS)-related lymphoma is generally divided into three types: systemic non-Hodgkin lymphoma (NHL), primary central nervous system lymphoma, and the primary effusion ("body cavity") lymphomas [1-3]. Systemic NHL accounts for the great majority of lesions [4].

Issues related to primary effusion lymphoma (PEL) will be reviewed here. The epidemiology of and risk factors of AIDS-related lymphoma and the other AIDS-related lymphomas are discussed separately. (See "AIDS-related lymphomas: Epidemiology, risk factors, and pathobiology" and "AIDS-related lymphomas: Clinical manifestations, diagnosis, and staging of systemic lymphoma" and "AIDS-related lymphomas: Treatment of systemic lymphoma" and "AIDS-related lymphomas: Primary central nervous system lymphoma".)


Primary effusion lymphoma (PEL) is one of the least common of the AIDS-related lymphomas, accounting for less than 1 to 4 percent of cases [4-6]. The overwhelming majority of cases of PEL occur in HIV-infected patients. However, this lesion can occur in the absence of HIV infection [7-10] and rarely has been seen following solid organ transplantation [11-13] and in chronic hepatitis C virus infection [14,15].

There appears to be a strong male predominance with men accounting for all 15 cases in one of the original descriptions, and for 10 of 11 cases in a separate single-institution study [4,7]. This may largely reflect the markedly increased prevalence of HIV infection among men. (See "AIDS-related lymphomas: Epidemiology, risk factors, and pathobiology" and "HIV infection and malignancy: Epidemiology and pathogenesis", section on 'Epidemiology'.)

Although earlier studies of this rare condition had reported low CD4 counts in patients with PEL [16], larger and more recent series do not support this observation [4,5,17]. Among those infected with HIV, patients with PEL are similar to those with other NHLs in age, race, and HIV transmission category [5].

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Literature review current through: Nov 2017. | This topic last updated: Sep 19, 2017.
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