Mohammad Farhan, Siddique Muhammad Neaman, Siddiqui Faraz, Popalzai M, Asgari Masoud, Odaimi Marcel
Department of Internal Medicine, Staten Island University Hospital, Staten Island, NY 10305, USA.
Department of Hematology-Oncology, Staten Island University Hospital, Staten Island, NY 10305, USA.
Case Rep Oncol Med. 2014;2014:436821. doi: 10.1155/2014/436821. Epub 2014 Mar 4.
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.
原发性渗出性淋巴瘤（PEL）或体腔淋巴瘤是一种罕见的B细胞起源的结外淋巴瘤，表现为淋巴瘤性渗出液，无任何淋巴结肿大或肿瘤肿块。它属于与艾滋病相关的非霍奇金淋巴瘤组。1996年首次在感染了卡波西肉瘤相关疱疹病毒（KSHV）或HHV-8病毒的HIV感染者中描述，2001年它被作为一个独立的实体纳入世界卫生组织造血和淋巴组织肿瘤分类中。该定义将与HHV-8病毒的关联作为一项强制性诊断标准。然而，后来有报道称在HHV-8阴性患者中诊断出了PEL样疾病过程。这最终被确认为一种罕见但独特的实体，称为“HHV-8无关的PEL样淋巴瘤”。在此，我们报告一例老年患者，该患者出现大量胸膜心包积液，最终被诊断为此种实体。迄今为止，仅报道了约50例HHV-8无关的PEL样淋巴瘤病例，而我们的病例EBV、HIV和丙型肝炎均为阴性，这使其非常独特且罕见。我们还对相关文献进行了综述，主要侧重于比较接受化疗和未接受化疗患者的治疗结果。
