Primary effusion lymphoma

Primary effusion lymphoma
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DOI:
10.1634/theoncologist.12-5-569
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发表时间:
2007-01-01
期刊:
影响因子:
5.8
通讯作者:
Hochberg, Ephraim
Hochberg, Ephraim
中科院分区:
医学2区
文献类型:
--
作者:
Chen, Yi-Bin;Rahemtullah, Aliyah;Hochberg, Ephraim

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原发性积液性淋巴瘤(PEL)是一种罕见的HIV相关非霍奇金淋巴瘤(NHL),约占所有HIV相关NHL的4%。PEL在体腔如胸膜间隙、心包和腹膜中有独特的临床表现。PEL细胞形态多变,淋巴细胞免疫表型为零,有人类疱疹病毒(HHV)-8感染的证据。HHV-8的确切致癌机制尚未明确。治疗通常是联合CHOP(环磷酰胺、阿霉素、长春新碱和强的松)化疗和抗逆转录病毒治疗(如果HIV阳性)。PEL的预后较差,中位生存时间约为6个月。随着HHV-8驱动肿瘤发生的确切分子步骤被揭开,人们希望能发现更具体的治疗靶点。
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.