A Unique Case of Malignant Pleuropericardial Effusion: HHV-8-Unrelated PEL-Like Lymphoma-A Case Report and Review of the Literature.
A Unique Case of Malignant Pleuropericardial Effusion: HHV-8-Unrelated PEL-Like Lymphoma-A Case Report and Review of the Literature.
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DOI：
10.1155/2014/436821
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发表时间：
2014
影响因子：
0.9
通讯作者：
Odaimi M
中科院分区：
文献类型：
--
作者：
Mohammad F;Siddique MN;Siddiqui F;Popalzai M;Asgari M;Odaimi M
原发性渗出性淋巴瘤（PEL）或体腔淋巴瘤是一种罕见的B细胞起源的淋巴结外淋巴瘤，表现为淋巴瘤性渗出，无任何淋巴结肿大或肿瘤肿块。它属于艾滋病相关的非霍奇金淋巴瘤。1996年首次在同时感染卡波西肉瘤相关疱疹病毒（KSHV）或HHV-8病毒的HIV感染个体中描述，2001年WHO将其作为造血和淋巴组织肿瘤分类中的一个单独实体。该定义包括与HHV-8病毒的关联作为强制性诊断标准。然而，后来报告了在HHV-8阴性患者中诊断出PEL样疾病过程的病例。这最终被认为是一种罕见但独特的实体，称为“HHV-8无关的PEL样淋巴瘤”。在此，我们报告一个老年患者谁提出了大量的胸膜心包积液，并最终被诊断为这种实体。到目前为止，只有大约50例HHV-8无关的PEL样淋巴瘤被报道，我们的病例是EBV，HIV和丙型肝炎阴性，这使得它非常独特和罕见。我们还对相关文献进行了综述，主要集中在比较接受化疗和不接受化疗患者的结局。
Primary effusion lymphoma (PEL) or body cavity lymphoma is a rare type of extra nodal lymphoma of B-cell origin that presents as lymphomatous effusion(s) without any nodal enlargement or tumor masses. It belongs to the group of AIDS related non-Hodgkin's lymphomas. First described in 1996 in HIV infected individuals who were coinfected with Kaposi's sarcoma-associated herpesvirus (KSHV) or HHV-8 virus, it was included as a separate entity in WHO classification of tumors of hematopoietic and lymphoid tissue in the year 2001. The definition included association with HHV-8 virus as a mandatory diagnostic criterion. However, cases were later reported where PEL-like disease process was diagnosed in HHV-8 negative patients. This was eventually recognized as a rare but distinct entity termed as “HHV-8-unrelated PEL-like lymphoma”. Herein, we are reporting a case of an elderly patient who presented with a large pleuropericardial effusion and was eventually diagnosed with this entity. Till date, only around 50 cases of HHV-8-unrelated PEL-like lymphoma have been reported and our case being EBV, HIV, and Hepatitis C negative makes it very unique and rare occurrence. We are also presenting a review of relevant literature focused mainly on comparing outcomes in patients treated with and without chemotherapy.