套细胞淋巴瘤患者的临床特征及预后因素分析

Clinical features and prognostic analysis of mantle cell lymphoma patients

目的 探讨套细胞淋巴瘤（MCL）患者的临床特征、疗效和预后影响因素。方法回顾性分析2003年8月至2013年6月中国医学科学院肿瘤医院收治的68例MCL患者的临床特征和不同治疗方案的疗效，并进行预后相关因素分析。结果68例MCL患者中位发病年龄58．5岁，男女比例为2．8：1，Ann Arbor分期以Ⅲ-Ⅳ期为主（占86．8％）。首发于淋巴结者56例，累及淋巴结外器官者49例，其中骨髓受侵19例。采用CHOP样方案化疗患者的客观缓解率为80．5％，明显高于采用加强方案化疗的患者（57．9％，P=0．036）。68例MCL患者的中位随访时间为35个月，3年和5年总生存率分别为78．5％和64．1％，中位无进展生存时间为20．0个月，2年和3年无进展生存率分别为41．3％和23．7％。单因素分析显示，Ⅲ-Ⅳ期、IPI评分为3～5分、Ki-67≥30％、乳酸脱氢酶（LDH）水平升高、β2微球蛋白水平升高、伴母细胞转化、受累淋巴结数≥5个、近期疗效评价为无效的患者无进展生存时间明显缩短。多因素分析显示，Ki-67指数、LDH水平和化疗疗效为影响患者无进展生存的独立预后因素。结论MCL多见于老年男性，发病率低，分期较晚，骨髓受累较常见。CHOP样方案治疗MCL比加强方案的客观有效率高，但预后仍较差。

Objective To analyze the clinical features, therapeutic outcome and prognostic factors of mantle cell lymphoma （MCL）. Methods Clinical data of a total of 68 patients with MCL admitted from August 2003 to June 2013 in our department were retrospectively analyzed. Results of all the patients, the median age was 58.5 years, with marked male predominance （2.8：1 ） , 59 patients （86.8%） were in Ann Arbor stage Ⅲ/Ⅳ. 56 cases （ 82.4% ） primarily showed lymph node involvement, 49 cased showed extranodal involvement and 19 cases （38.8%） had bone marrow involvement. Patients were followed up for 4 to 122 months with a median follow up time of 35 months. The 3- and 5-year overall survival （OS） rates were 78.5% and 64. 1%, respectively. The 2- and 3-year progression-free survival （PFS） rates were 41.3% and 23.7%, respectively, and the median time to progression was 20.0 months. The overall response rate （ORR） of CHOP regimen was superior to that of intense regimens （ P = 0. 036 ）. Univariate analysis showed that stage Ⅲ/Ⅳ, tPi score of 3-5, expression of Ki-67 higher than 30% , elevated LDH, elevated 132-MG, blastic variant, more than 5 lymph nodes involved, and failure to chemotherapy were the negative factors. Multivariate analysis showed that Ki-67 index, LDH and the response to chemotherapy were independent factors affecting survival. Conclusions Most patients with MCL were elderly males with advanced stage and usually had bone marrow involvement. Although ORR of CHOP regimen is superior to intense regimens, the prognosis of MCL remains poor.