原发性中枢神经系统淋巴瘤临床病理及病因学分析  

Clinical pathology and aetiology of primary central nervous system lymphoma

作　　者：周志毅[1] 程静[1] 石群立[1] 周晓军[1] 马恒辉[1] 周航波[1] 陆珍凤[1] 

机构地区：[1]南京大学医学院临床学院/南京军区南京总医院病理科,南京210002

出　　处：《临床与实验病理学杂志》2008年第2期211-214,218,共5页Chinese Journal of Clinical and Experimental Pathology

基　　金：江苏省六大高峰人才资助课题(2005A2)

摘　　要：目的探讨原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL)的临床病理特征、预后指标及病因学。方法复习39例PCNSL患者的临床资料,同时进行免疫组化、原位杂交检测EBER及PCR检测bcl-2/JH基因重排,并对临床资料、免疫标志物与预后的关系进行分析。结果34例PCNSL患者的3年生存率为46.4%,5年生存率为27.1%,年龄≥60岁及病变部位深对预后不利(P=0.009和P=0.027),bcl-6阳性表达者的生存率高于阴性表达者(P=0.044),但多因素Cox回归分析显示,进入回归方程的为年龄因素。CD10/bcl-6/MUM-1/CD138分型和治疗方法对预后的判断无显著性差异(P>0.05)。39例患者EBER原位杂交均为阴性,bcl-2/JH基因重排5例阳性(12.8%),其中3例为CD10阳性病例。结论PCNSL是一种少见的高侵袭性结外非霍奇金淋巴瘤,年龄因素是判断预后的独立性指标,CD10/bcl-6/MUM-1/CD138分型未发现有预后意义,但显示PCNSL的同质性较高,可能是弥漫性大B细胞淋巴瘤的一种亚型。EB病毒感染与PCNSL的病因无相关性。Purpose To investigate the clinical and pathological features, prognostic indicator and aetiology of primary central nervous system lymphoma （PCNSL）. Methods Clinical data of 39 cases of PCNSL were reviewed. In situ hybridization（ISH） and polymerase chain reaction （PCR） were used to detect EBER and bcl-2/JH gene rearrangement, and the relations of clinical data and immunological markers to the outcome were analyzed. Results The 3-year and 5-year survival rates of 34 patients were 46. 4% and 27.1% , unfavorable variables for survival rate were age older than 60 years and tumor location within deep region （ P = 0. 009 and P = 0. 027 ） , while expression of bcl-6 was favorable （ P =0. 044） , but only the factor of age entered the Cox regression model. Treatment and classification of CD10/bcl-6/MUM-1/CD138 were not relevant with prognosis （ P 〉 0. 05 ）. All patients were negative in EBER ISH. Five cases were positive in bcl-2/JH gene rearrangement, including all 3 CDlO-positive cases. Conclusions PCNSL is a rare aggressive extranodal non-Hodgkin＇ s lymphoma. Factor of age serves as an independent indicator for prognostic assessment, the typing of CD10/ bcl-6/MUM-1/CD138 is not a factor of prognosis, but suggesting that PCNSL is highly homogeneous, and possibly a subset of diffuse large B-cell lymphoma. EBV infection does not correlate with the aetiology of PCNSL.

关 键 词：中枢神经系统肿瘤 淋巴瘤 原位杂交 基因重排 预后 病因学 

分 类 号：R739.4[医药卫生—肿瘤] R733.4[医药卫生—临床医学]