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原发性中枢神经系统弥漫性大B细胞淋巴瘤表现为活化B细胞样表型,同时共表达C-MYC、BCL-2和BCL-6。

Primary central nervous system diffuse large B-cell lymphoma shows an activated B-cell-like phenotype with co-expression of C-MYC, BCL-2, and BCL-6.

作者信息

Li Xiaomei, Huang Ying, Bi Chengfeng, Yuan Ji, He Hong, Zhang Hong, Yu QiuBo, Fu Kai, Li Dan

机构信息

Department of Pathology, The First Affiliated Hospital, Chongqing Medical University, Chongqing 400016, China; Institute of Neuroscience, Faculty of Basic Medicine, Chongqing Medical University, Chongqing 400016, China.

Department of Pathology & Microbiology, University of Nebraska Medical Center, Omaha 68198, USA.

出版信息

Pathol Res Pract. 2017 Jun;213(6):659-665. doi: 10.1016/j.prp.2017.02.012. Epub 2017 Feb 24.

DOI:10.1016/j.prp.2017.02.012
PMID:28552541
Abstract

Diffuse large B-cell lymphoma (DLBCL) is the most common non-Hodgkin lymphoma, whose main prognostic factor is closely related to germinal center B-cell-like subtype (GCB- DLBCL) or activated B-cell-like type (non-GCB-DLBCL). The most common type of primary central nervous system lymphoma is diffuse large B-cell type with poor prognosis and the reason is unclear. This study aims to stratify primary central nervous system diffuse large B-cell lymphoma (PCNS-DLBCL) according to the cell-of-origin (COO) and to investigate the multiple proteins expression of C-MYC, BCL-6, BCL-2, TP53, further to elucidate the reason why primary central nervous system diffuse large B-cell lymphoma possesses a poor clinical outcome as well. Nineteen cases of primary central nervous system DLBCL were stratified according to immunostaining algorithms of Hans, Choi and Meyer (Tally) and we investigated the multiple proteins expression of C-MYC, BCL-6, BCL-2, TP53. The Epstein-Barr virus and Borna disease virus infection were also detected. Among nineteen cases, most (15-17 cases) were assigned to the activated B-cell-like subtype, highly expression of C-MYC (15 cases, 78.9%), BCL-2 (10 cases, 52.6%), BCL-6 (15 cases, 78.9%). Unfortunately, two cases were positive for PD-L1 while PD-L2 was not expressed in any case. Two cases infected with BDV but no one infected with EBV. In conclusion, most primary central nervous system DLBCLs show an activated B-cell-like subtype characteristic and have multiple expressions of C-MYC, BCL-2, BCL-6 protein, these features might be significant factor to predict the outcome and guide treatment of PCNS-DLBCLs.

摘要

弥漫性大B细胞淋巴瘤(DLBCL)是最常见的非霍奇金淋巴瘤,其主要预后因素与生发中心B细胞样亚型(GCB-DLBCL)或活化B细胞样型(非GCB-DLBCL)密切相关。原发性中枢神经系统淋巴瘤最常见的类型是弥漫性大B细胞型,预后较差,原因尚不清楚。本研究旨在根据起源细胞(COO)对原发性中枢神经系统弥漫性大B细胞淋巴瘤(PCNS-DLBCL)进行分层,并研究C-MYC、BCL-6、BCL-2、TP53的多种蛋白表达,进一步阐明原发性中枢神经系统弥漫性大B细胞淋巴瘤临床结局较差的原因。根据Hans、Choi和Meyer(计分法)的免疫染色算法对19例原发性中枢神经系统DLBCL进行分层,并研究C-MYC、BCL-6、BCL-2、TP53的多种蛋白表达。还检测了爱泼斯坦-巴尔病毒和博尔纳病病毒感染情况。在19例病例中,大多数(15 - 17例)被归为活化B细胞样亚型,C-MYC高表达(15例,78.9%)、BCL-2(10例,52.6%)、BCL-6(15例,78.9%)。遗憾的是,2例PD-L1呈阳性,而PD-L2在任何病例中均未表达。2例感染BDV,但无人感染EBV。总之,大多数原发性中枢神经系统DLBCL表现出活化B细胞样亚型特征,并具有C-MYC、BCL-2、BCL-6蛋白的多种表达,这些特征可能是预测PCNS-DLBCL结局和指导治疗的重要因素。

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