A clinicopathological study of primary central nervous system lymphomas & their association with Epstein-Barr virus

A clinicopathological study of primary central nervous system lymphomas & their association with Epstein-Barr virus
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DOI:
10.4103/0971-5916.187109
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发表时间:
2016-05-01
影响因子:
4.2
通讯作者:
Raina, Vinod
Raina, Vinod
中科院分区:
医学4区
文献类型:
--
作者:
Sharma, Mehar Chand;Gupta, Rakesh Kumar;Raina, Vinod

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背景与目的:原发性中枢神经系统淋巴瘤(PCNSLs)相对罕见,占原发性脑肿瘤的2- 3%。其中大多数是弥漫性大B细胞淋巴瘤(DLBCL),发生在免疫功能低下和免疫功能正常的患者中。本研究采用Hans分类法将PCNSL分为生发中心型(GC)和非生发中心型(NGC),并采用常规免疫组化(IHC)和显色原位杂交(CISH)方法探讨eb病毒(EBV)在PCNSL发病机制中的作用。方法:采用免疫组化检测CD45、CD20、CD3、b细胞淋巴瘤2和b细胞淋巴瘤6 (Bcl-2和Bcl-6)、b细胞特异性八聚体结合蛋白-1 (BOB-1)、多发性骨髓瘤癌基因-1 (mum1)、EBV潜伏膜蛋白1 (LMP-1)、cyclin-D1、CD10、CD5和CD23,以及CISH检测EBV。结果:10年间确诊PCNSL 65例,占颅内肿瘤的0.69%(65/9476)。平均发病年龄49岁,男女性别比(M:F)为1.4:1。最常见的部位是幕上区,主要累及额叶。单发病灶38例(58.4%),多发病灶27例(41.5%)。所有患者均未出现免疫功能低下。除1例滤泡性淋巴瘤外,所有病例均为B细胞免疫表型,均为DLBCL。根据Hans分类,多数为NGC型(n=51, 79.6%), 13例为GC型(20.3%)。34例(52.3%)肿瘤表达Bcl-2。EBV阳性3例(4.6%);2例同时经免疫组化和CISH检测,1例仅经CISH检测。解释与结论:在印度人群中,PCNSL主要发生在免疫功能正常的患者中,比西方人群早10年。免疫表型分析显示所有病例均为DLBCL,以NGC型为主。GC和NGC的DLBCL预后无差异。EBV的关联是罕见的,这种病毒可能不参与PCNSL在免疫正常个体的发病机制。CISH是一种简便、经济、省时的检测方法。
Background & objectives: Primary central nervous system lymphomas (PCNSLs) are relatively uncommon, accounting for 2-3 per cent of primary brain tumours. Majority of these are diffuse large B cell lymphomas (DLBCL) occurring both in immunocompromised and immunocompetent patients. We undertook this study to classify PCNSL into germinal centre (GC) and non-germinal centre (NGC) type based on Hans classification and to find the role of Epstein-Barr virus (EBV) in pathogenesis both by conventional immunohistochemistry (IHC) and chromogenic in situ hybridization (CISH).Methods: The consecutive cases of PCNSL during a 10 years period were analysed by IHC for CD45, CD20, CD3, B-cell lymphoma 2 and 6 (Bcl-2 and Bcl-6), B-cell specific octamer binding protein-1 (BOB-1), multiple myeloma oncogene-1 (MUM-1), EBV latent-membrane protein 1 (LMP-1), cyclin-D1, CD10, CD5 and CD23, as well as by CISH for EBV.Results: During a period of 10 years, 65 PCNSL were diagnosed which comprised 0.69 per cent (65/9476) of all intracranial tumours. The mean age of presentation was 49 yr with sex ratio (M:F) of 1.4:1. Most common location was supratentorial region with predominant involvement of frontal lobe. Single lesions were seen in 38 (58.4%) and multifocal lesions in 27 (41.5%) patients. None of the patients were immunocompromised. All cases were B cell immunophenotype and were DLBCL except one case of follicular lymphoma. According to Hans classification, majority of them were NGC (n=51, 79.6%) and 13 (20.3%) were GC type. Bcl-2 expression was noted in 34 (52.3%) tumours. EBV was positive in three (4.6%) cases; two were detected both by IHC and CISH and one case by CISH only.Interpretation & conclusions: In Indian population, PCNSL occurs mainly in immunocompetent patients, and a decade earlier than in western population. Immunophenotyping revealed that all cases were DLBCL with predominance of NGC type. No prognostic difference was seen between GC and NGC DLBCL. Association of EBV was rare and this virus was possibly not involved in the pathogenesis of PCNSL in immunocompetent individuals. CISH was an easy, economical and less cumbersome method for detection of EBV in PCNSL.