Munichor Mariana, Cohen Hector, Sarid Ronit, Manov Irena, Iancu Theodore C
Department of Pathology, Rambam Medical Center, Electron Microscopy Unit, Bruce Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel.
Acta Cytol. 2004 May-Jun;48(3):425-30. doi: 10.1159/000326398.
AIDS-related body cavity-based lymphoma, or primary effusion lymphoma (PEL), is a distinct clinicopathologic entity that occurs predominantly in immunosuppressed patients infected with human herpesvirus 8 (HHV-8), also known as Kaposi's sarcoma-associated herpesvirus. Although it rarely occurs in human immunodeficiency virus (HIV)-negative patients, we report such a case here.
A 74-year-old male, who was HIV and Epstein-Barr virus (EBV) negative, was admitted to the hospital with dyspnea and chest pain. Chest radiography and computed tomography showed right pleural effusion. Cytologic analysis of the pleural effusion revealed a high grade lymphoma with round nuclei, prominent nucleoli and abundant cytoplasm. Polymerase chain reaction performed on the pleural effusion was positive for HHV-8 and negative for EBV. On molecular studies, the immunoglobulin heavy and kappa light chains were rearranged. Flow cytometry revealed a hyperploid fraction with DNA index of 1.29 expressing CD30. Immunostaining for HHV-8 from a cell block was positive. Electron microscopy revealed lymphomalike cells, many in various stages of apoptosis, with large nucleoli and clusters of viruslike particles in the nucleoplasm.
A firm diagnosis of PEL can be established by the examination of cells from the lymphomatous effusion by a combination of cytology, molecular genetics, phenotypic features, immunostaining and electron microscopy. To our knowledge, this is the first case in which immunostaining for anti-HHV-8 monoclonal antibodies was used to support the diagnosis.
艾滋病相关体腔淋巴瘤,即原发性渗出性淋巴瘤(PEL),是一种独特的临床病理实体,主要发生于感染人类疱疹病毒8型(HHV-8,又称卡波西肉瘤相关疱疹病毒)的免疫抑制患者。尽管其在人类免疫缺陷病毒(HIV)阴性患者中罕见,但我们在此报告一例。
一名74岁男性,HIV及爱泼斯坦-巴尔病毒(EBV)均为阴性,因呼吸困难和胸痛入院。胸部X线及计算机断层扫描显示右侧胸腔积液。胸腔积液的细胞学分析显示为高级别淋巴瘤,细胞核圆形,核仁明显,细胞质丰富。对胸腔积液进行的聚合酶链反应检测显示HHV-8阳性,EBV阴性。分子研究显示免疫球蛋白重链和κ轻链发生重排。流式细胞术显示有一个DNA指数为1.29的超二倍体部分表达CD30。细胞块的HHV-8免疫染色呈阳性。电子显微镜检查发现淋巴瘤样细胞,许多处于不同凋亡阶段,有大核仁,核质中有病毒样颗粒簇。
通过对淋巴瘤性渗出液中的细胞进行细胞学、分子遗传学、表型特征、免疫染色及电子显微镜检查相结合,可明确诊断PEL。据我们所知,这是首例使用抗HHV-8单克隆抗体免疫染色来支持诊断的病例。