ICD-O M9678/3 MeSH D054685 | DiseasesDB 33904 | |
Specialty Hematology and oncology | ||
Primary effusion lymphoma (PEL) is a B-cell lymphoma, presenting with a malignant effusion without a tumor mass.
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Causes
PEL most commonly arises in patients with underlying immunodeficiency, such as AIDS. PEL is caused by Kaposi's sarcoma-associated herpesvirus (KSHV), also known as human herpesvirus 8 (HHV-8). In most cases, the lymphoma cells are also infected with Epstein Barr virus (EBV).
The condition can exist in the absence of HHV-8 and HIV, though this is rare.
Presentation
PEL is unusual in that the majority of cases arise in body cavities, such as the pleural space or the pericardium; another name for PEL is "body cavity lymphoma".
The immunophenotype : CD45+ (95%), CD20-, CD79a-, PAX5-, CD30+, CD38+, CD138+ and EMA+. Staining the nucleus for HHV-8 LANA may be helpful.
History
It was recognized as a unique type of lymphoma only after the discovery of KSHV in 1994.
Prognosis
It is generally resistant to cancer chemotherapy drugs that are active against other lymphomas, and carries a poor prognosis.
Sirolimus has been proposed as a treatment option.