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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 [1,2]. Systemic NHL accounts for the great majority of lesions [3].

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 acquired immune deficiency syndrome (AIDS)-related lymphomas, accounting for less than 1 to 4 percent of cases [3-5]. The overwhelming majority of cases of PEL occur in human immunodeficiency virus (HIV)-infected patients. However, this lesion can occur in the absence of HIV infection [6-9] and rarely has been seen following solid organ transplantation [10-12] and in chronic hepatitis C virus infection [13,14].

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 [3,6]. 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 [15], larger and more recent series do not support this observation [3,4,16]. Among those infected with HIV, patients with PEL are similar to those with other NHLs in age, race, and HIV transmission category [4].


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Literature review current through: Sep 2016. | This topic last updated: Jan 19, 2016.
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