Primary effusion lymphoma.
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.
Chen
Yi-Bin
YB
Dana-Faber Cancer Institute, Boston, Massachusetts, USA.
Rahemtullah
Aliyah
A
Hochberg
Ephraim
E
D044966:Anti-Retroviral Agents / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001202:Ascitic Fluid / Q000821:virology; D003520:Cyclophosphamide / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D015658:HIV Infections / Q000150:complications / Q000188:drug therapy; D006566:Herpesviridae Infections / Q000150:complications / Q000188:drug therapy; D019288:Herpesvirus 8, Human; D006801:Humans; D008228:Lymphoma, Non-Hodgkin / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology / Q000821:virology; D009367:Neoplasm Staging; D010490:Pericardial Effusion / Q000188:drug therapy / Q000821:virology; D010996:Pleural Effusion / Q000188:drug therapy / Q000821:virology; D011241:Prednisone / Q000627:therapeutic use; D014750:Vincristine / Q000627:therapeutic use
D044966:Anti-Retroviral Agents; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone