Affiliation:
1. a Dana-Farber Cancer Institute, Boston, Massachusetts, USA
2. b Massachusetts General Hospital, Boston, Massachusetts, USA
Abstract
Abstract
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.
Publisher
Oxford University Press (OUP)
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