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WHO Classification of Tumours
Primary effusion lymphoma
Pleura, NOS


Definition

Primary effusion lymphoma (PEL) is a large B-cell neoplasm, presenting as a serous effusion without detectable tumor masses. It is universally associated with human herpes virus 8 (HHV-8)/Kaposi sarcoma herpes virus (KSHV). The most common sites of involvement are the pleural, pericardial, and peritoneal cavities
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Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



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The majority of cases arise in young or middle-aged homosexual or bisexual males with human immunodeficiency virus (HIV) infection and severe immunodeficiency. The neoplastic cells are positive for HHV8 and often co-infected with EBV. PEL also occurs in recipients of solid organ transplants in the absence of immunodeficiency, usually in elderly patients from areas with high prevalence for HHV8 infection such as the Mediterranean.
The most common sites are the pleural, pericardial and peritoneal cavities. Typically, only one body cavity is involved. The prognosis is unfavorable.