陈瑢，韩敬力，师晓莉，马志萍，李新霞( 新疆医科大学第一附属医院，乌鲁木齐830054;新疆医科大学第六附属医院)

原发中枢神经系统弥漫大B细胞淋巴瘤的临床病理特征及预后分析

陈瑢1，韩敬力2，师晓莉1，马志萍1，李新霞1
( 1新疆医科大学第一附属医院，乌鲁木齐830054;2新疆医科大学第六附属医院)

摘要:目的探讨原发中枢神经系统弥漫大B细胞淋巴瘤( PCNS-DLBCL)患者的临床病理特征及预后情况。方法选择手术切除的PCNS-DLBCL组织30份，采用HE染色及EnVision二步法观察PCNS-DLBCL组织病理学特征和免疫表型特征;单因素分析患者发病年龄、国际预后指数( IPI)评分、体能状态( PS)评分、治疗方式、免疫表型与预后的关系，并采用COX回归模型对影响患者预后的因素进行多因素分析。结果PCNS-DLBCL患者的临床表现以颅内压升高、局灶性神经功能缺损为主;形态学以中心母细胞型为主;免疫表型以non-GCB型为主。单因素分析显示，发病年龄、IPI评分、PS评分、治疗方式、免疫表型与患者预后有关( P均＜0.05) ;多因素分析显示，IPI评分≥2分与预后相关( P＜0.05)。结论PCNS-DLBCL临床表现无特异性，患者长期生存率低，预后较差; IPI评分与患者预后有关。

关键词:淋巴瘤，大细胞，弥漫型;中枢神经系统;免疫表型;预后

Clinical and pathological features of primary central nervous system diffuse large B-cell lymphoma

CHEN Rong1，HAN Jing-li，SHI Xiao-li，MA Zhi-ping，LI Xin-xia
( 1 The First Affiliated Hospital of Xinjiang Medical University，Urumqi 830054，China)

Abstract:Objective To investigate the clinical and pathological features of primary central nervous system diffuse large B-cell lymphoma ( PCNS-DLBCL)，and to investigate survival situation.Methods We got 30 cases of tissue specimen by surgical resection from PCNS-DLBCL patients，then observed pathological characteristics and immune phenotype by using EnVision two steps and HE staining.Univariate analysis was used to detect the relationships between prognosis and the features including age，PS score，IPI score，treatment and immune subtype.The Cox regression model analysis was used to analyze multivariate influence factors.Results The clinical manifestations were mainly intracranial high pressure and focal neurological deficits.Tumor cells mostly were centroblasts in morphology.The immune phenotype was mainly non-GCB type.Univariate analysis showed that the age，PS score，IPI score，treatment and immune subtype were statistically related to the prognosis of patients ( all P＜0.05).Multivariate analysis showed that IPI score which was more than 2 was related to poor prognosis ( P＜0.05).Conclusions The clinical manifestations of PCNS-DLBCL were nonspecific，the long-term survival rate was low and the prognosis was poor.The IPI score was related to the prognosis of patients.

Key words:lymphoma，large cell，diffuse; central nervous system; immune phenotype; prognosis

原发中枢神经系统弥漫大B细胞淋巴瘤( PCNS-DLBCL)是最常见的原发中枢神经系统淋巴瘤( PCNSL)，近年来其发病率有逐年升高趋势。2008 年WHO关于淋巴造血系统肿瘤分类中将PCNSDLBCL作为非特指的弥漫大B细胞淋巴瘤( DLBCL)中的一个亚型单独列出［1］。临床研究发现，PCNS-DLBCL在临床表现、形态学特点、免疫表型和遗传学特征等方面存在很大的异质性。……