Tsagarakis Nikolaos J, Argyrou Aspasia, Gortzolidis Georgios, Kentrou Nektaria, Papadhimitriou Stefanos I, Tzanetou Konstantina, Kakiopoulos Georgios, Papadimitriou Konstantinos A, Skoumi Dimitra, Paterakis Georgios
Flow Cytometry Laboratory, Department of Immunology, Athens Regional General Hospital "G. Gennimatas", Mesogion Avenue 154, 11527, Athens, Greece.
Department of Clinical Hematology, Athens Regional General Hospital "G. Gennimatas", Athens, Greece.
Int J Hematol. 2009 Jul;90(1):94-98. doi: 10.1007/s12185-009-0343-0. Epub 2009 May 30.
Although primary effusion lymphoma (PEL) is usually associated with human herpes virus-8/Kaposi sarcoma herpes virus (HHV-8/KSHV) and human immunodeficiency virus (HIV), there are several reports of HHV-8/KSHV and HIV negative cases, mainly in the setting of immunodeficiency. Here, we report the second case of PEL associated with idiopathic T4 lymphocytopenia (ICL), which was HHV-8/KSHV negative, HIV negative and Epstein-Barr virus positive, while no other causative agents for immunodeficiency were documented. Flow cytometry revealed a hyperdiploid and highly mitotic large B-cell population, CD30, EMA, CD66, CD38 and CD71 positive. The malignant lymphoma cells showed atypia with prominent nuclei and basophilic vacuolated cytoplasm, while cytogenetic analysis with fluorescent in situ hybridization showed trisomy 18. The patient was administered R-COP chemotherapy, but no remission was achieved, up to 3 months from diagnosis.
尽管原发性渗出性淋巴瘤（PEL）通常与人类疱疹病毒8型/卡波西肉瘤疱疹病毒（HHV-8/KSHV）和人类免疫缺陷病毒（HIV）相关，但有几篇关于HHV-8/KSHV和HIV阴性病例的报道，主要发生在免疫缺陷的情况下。在此，我们报告第二例与特发性T4淋巴细胞减少症（ICL）相关的PEL病例，该病例HHV-8/KSHV阴性、HIV阴性且EB病毒阳性，同时未记录到其他免疫缺陷的致病因素。流式细胞术显示一个超二倍体且有高度有丝分裂活性的大B细胞群体，CD30、EMA、CD66、CD38和CD71呈阳性。恶性淋巴瘤细胞表现出异型性，细胞核突出，胞质嗜碱性且有空泡，而荧光原位杂交的细胞遗传学分析显示18号染色体三体。该患者接受了R-COP化疗，但从诊断起3个月内未实现缓解。
