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The Oncologist, Vol. 12, No. 5, 569-576, May 2007; doi:10.1634/theoncologist.12-5-569
© 2007 AlphaMed Press

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Lymphoma

Primary Effusion Lymphoma

Yi-Bin Chena, Aliyah Rahemtullahb, Ephraim Hochbergb

aDana-Farber Cancer Institute, Boston, Massachusetts, USA; bMassachusetts General Hospital, Boston, Massachusetts, USA

Correspondence: Ephraim Hochberg, M.D., Yawkey 7B-7854, MGH, 32 Fruit Street, Boston, Massachusetts 02114, USA. Telephone: 617-726-8743; Fax: 617-643-1915; e-mail: ehochberg{at}partners.org

Primary effusion lymphoma (PEL) is a rare HIV-associated non-Hodgkin's lymphoma (NHL) that accounts for approximately 4% of all HIV-associated NHL. PEL has a unique clinical presentation in having a predilection for arising in body cavities such as the pleural space, pericardium, and peritoneum. PEL cells are morphologically variable with a null lymphocyte immunophenotype and evidence of human herpesvirus (HHV)-8 infection. The exact oncogenic mechanisms of HHV-8 have not been clearly defined. Treatment is usually with combination CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy and antiretroviral therapy (if HIV positive). The prognosis for PEL is poor, with a median survival time of around 6 months. As the exact molecular steps in HHV-8–driven oncogenesis are unraveled, it is hoped that more specific therapeutic targets will be revealed.

Disclosure of potential conflicts of interest is found at the end of this article.




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