Primary effusion lymphoma
| Primary effusion lymphoma | |
| ICD-O: | 9678/3 |
|---|---|
| DiseasesDB | 33904 |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords: Body cavity lymphoma
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)[1]. 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"[2].
Historical Perspective
Primary effusion lymphoma was recognized as a unique type of lymphoma only after the discovery of KSHV in 1994.
Causes
PEL most commonly arises in patients with underlying immunodeficiency, such as AIDS[3]. It is caused by Kaposi's sarcoma-associated herpesvirus (KSHV).
Prognosis
PEL carries a very poor prognosis.
Treatment
Primary effusion lymphoma is generally resistant to cancer chemotherapy drugs that are active against other lymphomas [4].
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.
- ↑ 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.
- ↑ 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.