80例原发中枢神经系统弥漫大B细胞淋巴瘤的临床病理、免疫表型及EB病毒感染的研究

目的:对80例原发中枢神经系统弥漫大B细胞淋巴瘤（primary diffuse large B cell lymphoma of the central nervous system,PCNS DLBCL）进行临床病理学回顾性研究、免疫表型检测及EB病毒（Epstein-Barr virus,EBV）感染检测。旨在探讨其与预后的关系。方法:对80例PCNS DLBCL进行免疫表型检测及EB病毒检测，并进行Hans、Choi和Tally分型、统计学单因素和多因素预后分析。结果:Bcl-2、CD10、Bcl-6、Mum-1、GCET-1、BLIMP-1、FOXP-1和LMO-2的表达率分别为46.1%、8.8%、75.0%、57.5%、27.5%、11.3%、75.0%和26.3%；Ki-67指数为30%～95%，中位数为80%。Hans、Choi和Tally分型中Non-GCB型/ABC型弥漫大B细胞淋巴瘤（diffuse large B cell lymphoma,DLBCL）（63.9%、79.2%和90.0%）为最常见的亚型。EBV及EBER1/2-ISH的表达率均为3.8%。58.8%的患者术后未行其它治疗，其1年、2年和5年生存率分别为36.3%、16.4%和4.6%。术后是否行其它治疗、采用甲氨喋呤（methotrexate,MTX）治疗和环磷酰胺+多柔比星+长春新碱+泼尼松龙（cyclophosphamide,doxorubicin,vincristine,and prednisone,CHOP）治疗是有统计学意义的预后相关因素（P均＜0.001）。结论:80例PCNS DLBCL患者年龄较国内外报道的小；以Non-GCB型/ABC型DLBCL为主；个别病例检出EB病毒感染；术后未行其它治疗组、未采用MTX治疗组和未CHOP治疗组的预后较差。

Primary diffuse large B cell lymphoma of the central nervous system:A study of 80 cases of in clinicopathology,immunophenotype and Epstein Barr virus infection

Objective:80 cases of PCNS DLBCL were retrospectively investigated,including clinical manifestations,histopathology,immunophenotype,as well as detection of Epstein-Barr virus (EBV) in this study.The aim is to summarize the clinical and pathologic features of the tumor,to evaluate the significances of immunophenotype and its role in sub-classification of the tumor,to observe the EBV status.Methods:Staining for CD20,CD3,CD10,Bcl-6,Mum-1,CD5,Bcl-2,Ki-67,FOXP-1,GCET-1,BLIMP-1,LMO-2 and EBV antigens were performed on 80 paraffin-embedded sections.Hans,Choi and Tally type were classified respectively,then their prognosis were analyzed using statistical univariate and multivariate.Immunopheno-type analysis was performed by both EnVision and EliVision method.In situ hybridization (ISH) was carried out to detect small molecular RNA encoded by EBV.Results:The expression of Bcl-2,CD10,Bcl-6,Mum-1,GCET-1,BLIMP-1,FOXP-1 and LMO-2 were 46.1%,8.8%,75.0%,57.5%,27.5%,11.3%,75.0% and 26.3% respectively.Ki-67 index ranged from 30% to 95%,with a median of 80%.Based on Hans,Choi and Tally classifications,Non-GCB type/ABC type DLBCL was the most common subtype (63.9%,79.2% and 90.0%).The tumor cells gave positive reaction to both EBV protein and EBER1/2-ISH in 3.8% of the cases.58.8% of the patients had not received any other therapy following excision of the tumor.The overall survival (OS) of 1,2 and 5 years was 36.3%,16.4% and 4.6% respectively.Statistical analysis showed that the following three agents were the prognosis related factors,including the further treatment after the operation,usage of methotrexate (MTX) and CHOP regimens (P＜0.001).Conclusion:In present study,the median age of the patients was younger than that reported in literature.Non-GCB type/ABC type DLBCL was predominant.EBV infection was detected in individual cases.In patients with no further therapy accepted after surgery,without use MTX and CHOP regimen,the prognosis was poor.