Primary effusion lymphoma

Primary effusion lymphoma
ICD-O:	9678/3
DiseasesDB	33904

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Primary effusion lymphoma (PEL) is a malignancy of B cells that is caused by Kaposi's sarcoma-associated herpesvirus (KSHV), also known as human herpesvirus 8 (HHV-8). In about 80% of cases, the lymphoma cells are also infected with Epstein Barr virus (EBV). 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". It was recognized as a unique type of lymphoma only after the discovery of KSHV in 1994.

PEL most commonly arises in patients with underlying immunodeficiency, such as AIDS. It is generally resistant to cancer chemotherapy drugs that are active against other lymphomas, and carries a very poor prognosis.

References

• Cesarman E, Chang Y, Moore PS, Said JW, Knowles DM. Kaposi's sarcoma-associated herpesvirus-like DNA sequences in AIDS-related body-cavity-based lymphomas. N Engl J Med 1995;332(18):1186-91.
• Boshoff C, Weiss R. AIDS-related malignancies. Nat Rev Cancer 2002;2(5):373-82.
• Yarchoan R, Tosato G, Little RF. Therapy insight: AIDS-related malignancies - the influence of antiviral therapy on pathogenesis and management. Nature Clin Prac Oncology 2005;2(8):406-15.
• Staudt MR, Kanan Y, Jeong JH, Papin JF, Hines-Boykin R, Dittmer DP. The tumor microenvironment controls primary effusion lymphoma growth in vivo. Cancer Res 2004;64(14):4790-9.

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