原发中枢神经系统淋巴瘤20例临床特点与预后分析

Clinical Characteristics and Prognosis of 20 Cases of Primary Central Nervous System Lymphoma

目的:本研究探讨原发性中枢神经系统淋巴瘤的临床表现、病理特征、疗效及预后。方法:回顾性分析20例原发性中枢神经系统淋巴瘤的临床表现、病理学特征、诊治、疗效、生存情况。结果:临床表现头痛、偏瘫、癫痫、头晕、智力减退等;脑内单发病灶7例,多发病灶13例;病理类型:外周T细胞淋巴瘤1例,弥漫大B细胞淋巴瘤(DLBCL)19例,均为非生发中心来源;细胞增殖指数Ki-67>75%11例,50%-75%6例,25%-50%2例,10%1例;18例接受治疗的患者治疗后评价CR 10例、PR 2例、PD 6例;治疗过程中是否联用利妥昔单克隆抗体以及颅内病灶数多少与患者的生存时间明显相关(P<0.05);Ki-67(+)<75%病例生存时间长于Ki-67(+)>75%病例(P=0.042);年龄和脑脊液蛋白高低对疗效的影响差异有显著性(P<0.05)。结论:PCNSL的临床表现、体征以及影像学特点均缺乏特异性,确诊需要有病理活检;年龄>60岁、脑脊液蛋白>600 mg/L、发病时有颅内多发病灶、Ki-67(+)>75%是预后不良因素。PCNSL恶性程度高,预后差,大剂量甲氨蝶呤(HD-M TX)为基础的化疗(其中DLBCL患者联用利妥昔单克隆抗体)并在完全缓解后采用造血干细胞移植是目前治疗PCNSL临床首选的治疗方案。

Objective：This study was to investigate the clinical and pathological characteristics, curative effect and prognosis of primary central nervous system lymphoma （PCNSL）. Methods：The clinical and pathological features, diagnosis and treatment, curative efficacy and survival rate of 20 cases of primary central nervous system lymphoma were retrospectivaly analyzed. Results：The indicated that the clinical manifestations included headache, paralysis, seizures, dizziness and mental acuity, etc. There were 7 cases with single focus of lesion, 13 cases with multiple foci of lesions. The pathologic examimation showed 1 case of peripheral T-cell lymphoma and 19 cases of diffuse large B cell lymphoma, they all were from non-germinal center. Out of 20 cases, 11 cases with Ki-67 〉75% ,6 cases with Ki-67 50% -75%, 2 cases with Ki-67 25% -50% and 1 case with Ki-67 10%. Therapeutic evaluation were as follows： 10 cases with CR, 2 cases with PR, 6 cases with PD in 18 patients recieved treatment. The use of ritusimab in treatment or no use and number of lesion foci in cranium were related to survival time （ P 〈 0.05 ）. The survival time in group with Ki-67（ ＋ ） 〈75% was longer than that in group with Ki-67 （ ~ ） 〉75% （P =0. 042）. Age and CSF-TP level were related to curative effect （ P 〈 0. 05 ）. Conclution： There is no specific clinical manifestation. The physical examination and imageological diagnosis of PCNSL, as well as pathological biopsy are necessary for diagnose of PCNSL. The factors for poor prognosis include age 〉 60 years, CSF-TP 〉 600 rag/L, multiple lesions and Ki-67（ ＋ ） 〉75%. PCNSL has high malignancy and poor prognosis. The chemotherapy based on high dose of MTX （ among which MTX combined with rituximab） and HSCT after CR are the best treatment regimen for DLBCL.