原发性中枢神经系统淋巴瘤167例临床特征及病理类型构成分析  

Analysis of clinical features,pathological types and composition of primary central nervous system lymphoma: a report of 167 cases

作　　者：李丽[1,2] 谢建兰[1] 张燕林[1] 郑媛媛[1] 周小鸽[1] LI Li;XIE Jian-lan;ZHANG Yan-lin;ZHENG Yuan-yuan;ZHOU Xiao-ge(Department of Pathology,Beijing Friend Hospital,Capital Medical University,Beijing 100050,China;Department of Pathology,Erdos Central Hospital,Erdos 017000,China)

机构地区：[1]首都医科大学附属北京友谊医院病理科,北京100050 [2]鄂尔多斯市中心医院病理科,鄂尔多斯017000

出　　处：《临床与实验病理学杂志》2018年第11期1189-1193,共5页Chinese Journal of Clinical and Experimental Pathology

基　　金：国家自然科学基金(81272633)

摘　　要：目的探讨原发性中枢神经系统淋巴瘤(primary central nervous system lymphomas,PCNSL)的临床特征、病理类型及构成比率,分析PCNSL的EBV感染率、c-MYC、BCL-2及BCL-6基因异常及免疫球蛋白基因重排情况。方法回顾性分析167例PCNSL的临床病理资料,总结其临床特征、病理类型及构成比率;原位杂交技术检测PCNSL中EBV编码的小RNA(EBER);荧光原位杂交(fluorescence in situ hybridization,FISH)技术检测c-MYC、BCL-2、BCL-6基因扩增及断裂重排情况;免疫球蛋白基因重排检测Ig H和Ig K。结果 PCNSL占所有淋巴瘤的0. 95%;大多为单一病灶,约占60%,且多位于浅部脑组织(66. 93%)及小脑幕上(87. 40%),最常累及额叶和颞叶,51～60岁为发病高峰期。PCNSL中霍奇金淋巴瘤(Hodgkin’s lymphoma,HL)仅1例;非霍奇金淋巴瘤(non-Hodgkin’s lymphoma,NHL) 166例:B细胞淋巴瘤占158例,其中弥漫大B细胞淋巴瘤(diffuse large B-cell lymphoma,DLBCL)最常见; T/NK细胞淋巴瘤8例,其中以ALK阳性间变大细胞淋巴瘤多见。13例原发性中枢神经系统弥漫大B细胞淋巴瘤(primary central nervous system-diffuse large B-cell lymphoma,PCNS-DLBCL) EBER均阴性; 11例PCNS-DLBCL中1例c-MYC基因拷贝数增加,未见断裂重排; 1例BCL-2基因拷贝数增加,未见断裂重排; 2例BCL-6基因拷贝数增加,1例断裂重排;免疫球蛋白Ig H和Ig K呈克隆性重排。结论 PCNSL病理类型多样,HL和NHL均可发生,以PCNSDLBCL最多见,T细胞来源的淋巴瘤以ALK阳性间变大细胞淋巴瘤多见,51～60岁为发病高峰期。Purpose To study the clinical/pathological characteristics and composition proportion of primary central nervous system lymphomas( PCNSL). To analyzed the EBV infection rate,c-MYC,BCL-2 and BCL-6 gene abnormality and immunoglobulin gene rearrangement of PCNSL. Methods Clinical data of 167 PCNSL cases during 2007 and 2017 were retrospectively analyzed,and the basic data,clinical/pathological characteristics,as well as composition proportion were summarized. In situ hybridization was used to detect small RNA encoded by EB virus( EBER) in PCNSL. Fluorescence in situ hybridization was used to detect c-MYC,BCL-2,BCL-6 gene amplification and fracture rearrangement. Immunoglobulin gene rearrangement was used to detect Ig H and Ig K. Results Most of PCNSLs were single lesion( 60%),66. 93% and 87. 40% PCNSL located in shallow brain tissue and tentorium cerebelli,and mostly involved the frontal and temporal lobes,the peak of onset was 51-60 years old. In this study,PCNSL included 17 pathological types,including 1 case of Hodgkin’s lymphoma,166 cases of non-Hodgkin’s lymphoma. Among non-Hodgkin’s lymphoma,158 cases were of the B-cell lymphoma which the majority were diffuse large B-cell lymphoma. T/NK cell lymphoma was seen in 8 cases,especially anaplastic lymphoma kinase( ALK) positive anaplastic large cell lymphoma. All of the 13 cases of PCNS-DLBCL were negative for EBV-encoded small RNA. In 11 cases of PCNS-DLBCL,c-MYC gene copy number increased in 1 case,and no fracture rearrangement was found. 1 case of BCL-2 gene copy number increased,no fracture rearrangement was found. 2 cases of BCL-6 gene copy number had increased,and 1 case had broken rearrangement. Ig H and Ig K were clonal rearrangement. Conclusion PCNSL has a variety of pathological types,both the Hodgkin’s lymphoma and nonHodgkin’s lymphoma can occur. Among all the PCNSL and Bcell PCNSL,diffuse large B-cell lymphoma is the most common type of lymphoma,ALK-positive anaplastic large cell lymphoma is the most common type of T-cell lymphoma. The peak o

关 键 词：淋巴瘤 弥漫大B细胞淋巴瘤 免疫组织化学 

分 类 号：R733.4[医药卫生—肿瘤]