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心包内与人类疱疹病毒8无关的原发性渗出性淋巴瘤样淋巴瘤:1例伴有Ⅲ型潜伏性EB病毒感染且未经化疗预后良好的病例

Human herpes virus 8-unrelated primary effusion lymphoma-like lymphoma in the pericardium: A case with latency type III Epstein-Barr virus infection showing good prognosis without chemotherapy.

作者信息

Nakamura Harumi, Tsuta Koji, Nakagawa Takashi, Hirai Risen, Ota Yasunori

机构信息

Department of Pathology, National Center for Global Health and Medicine Hospital, Tokyo, Japan.

Department of Clinical Laboratories, Kansai Medical University Hirakata Hospital, Osaka, Japan.

出版信息

Pathol Res Pract. 2015 Dec;211(12):1010-3. doi: 10.1016/j.prp.2015.08.002. Epub 2015 Sep 5.

Abstract

Primary effusion lymphoma (PEL) is a rare subtype of non-Hodgkin lymphoma that proliferates in body cavities without detectable masses. PEL is universally associated with human herpes virus-8 (HHV-8) infection and has an aggressive prognosis. Recently, an HHV-8-unrelated PEL-like lymphoma that usually occurs in elderly individuals and follows a more indolent prognosis has been reported, and it is treated as a disease distinct from PEL. However, its pathogenesis and prognostic factors have not been sufficiently clarified. In PEL-like lymphoma accompanied by Epstein-Barr virus (EBV) infection, latent infection types are not mentioned in the literature. Herein, we report the case of an 85-year-old Japanese man with pericardial PEL-like lymphoma who showed good improvement in condition for 24 months after pericardiocentesis without chemotherapy. Serological test results were positive for EBV capsid antigen and EBV nuclear antigen 2 (EBNA2), but negative for human immunodeficiency virus, hepatitis B virus, and hepatitis C virus. The disease phenotype and EBV infection mechanism were immunohistochemically investigated by the cellblock prepared from pericardial effusion. Atypical cells were positive for CD20, CD30, CD45, BCL2, MUM1, EBNA2, latent membrane protein 1, and EBV-encoded RNA (on in situ hybridization), but negative for CD3, CD5, CD10, CD138, cytokeratin AE1/AE3, and HHV-8. Accordingly, this case was considered to be a B-cell activated phenotype with a type III latent EBV infection. Type III latent EBV infection is unusual in PEL.

摘要

原发性渗出性淋巴瘤(PEL)是一种罕见的非霍奇金淋巴瘤亚型,在体腔内增殖且无明显肿块。PEL普遍与人类疱疹病毒8型(HHV-8)感染相关,预后较差。最近,有报道称一种与HHV-8无关的PEL样淋巴瘤,通常发生于老年人,预后相对惰性,被视为一种与PEL不同的疾病。然而,其发病机制和预后因素尚未完全阐明。在伴有爱泼斯坦-巴尔病毒(EBV)感染的PEL样淋巴瘤中,文献中未提及潜伏感染类型。在此,我们报告一例85岁日本男性心包PEL样淋巴瘤病例,该患者在未进行化疗的心包穿刺术后病情改善良好,持续24个月。血清学检测结果显示EBV衣壳抗原和EBV核抗原2(EBNA2)呈阳性,但人类免疫缺陷病毒、乙型肝炎病毒和丙型肝炎病毒呈阴性。通过心包积液制备的细胞块对疾病表型和EBV感染机制进行了免疫组织化学研究。非典型细胞CD20、CD30、CD45、BCL2、MUM1、EBNA2、潜伏膜蛋白1和EBV编码RNA(原位杂交阳性)呈阳性,但CD3、CD5、CD10、CD138、细胞角蛋白AE1/AE3和HHV-8呈阴性。因此,该病例被认为是具有III型潜伏EBV感染的B细胞活化表型。III型潜伏EBV感染在PEL中并不常见。

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