血管免疫母细胞性T细胞淋巴瘤42例临床分析

Angioimmunoblastic T cell lymphoma： clinical analysis of 42 cases

目的 分析血管免疫母细胞性T细胞淋巴瘤（AITL）的临床特征及预后因素.方法 回顾性分析2007年1月至2012年8月在北京大学肿瘤医院住院治疗的初治42例AITL患者的临床资料,总结其临床特征并分析其预后因素.结果 42例患者的中位年龄为59岁（年龄范围34 ～ 76岁）,其中97.6％ （41/42）为Ⅲ～Ⅳ期患者,73.8％ （31/42）伴有B症状;85.7％（36/42）以无痛性浅表淋巴结肿大起病;52.4％（22/42）出现结外受侵;64.3％（27/42）起病时乳酸脱氢酶升高;45.2％（19/42）β2-微球蛋白升高;淋巴瘤国际预后指数（IPI）评分达3分即中高危组所占比例最高,占40.5％ （17/42）.一线治疗主要采用CHOP（环磷酰胺、阿霉素、长春新碱、泼尼松）类方案化疗,44.7％（17/38）获完全缓解.中位随访时间40个月（2～106个月）.1、2和5年生存率分别为78％、57％和39％.统计学分析显示IPI评分为患者独立预后因素（P =0.009）,其余因素包括性别（P=0.311）、年龄（P=0.263）、是否伴有B症状（P =0.102）、Ki-67指数（P =0.146）以及一线化疗方案的选择（P=0.292）均有影响生存的趋势,但尚未具有统计学意义.结论 AITL发病年龄偏大,分期晚,预后不佳.IPI评分为患者独立预后因素,患者的性别、年龄、是否伴有B症状、Ki-67指数以及一线化疗方案的选择可能有影响生存的趋势.

Objective To explore the clinical characteristics and prognosis of patients with angioimmunoblastic T cell lymphoma （AITL）.Methods The clinical features and prognostic factors of 42 cases newly diagnosed as AITL at Peking University Cancer Hospital from January 2007 to August 2012 were retrospectively analyzed.Results Their median age was 59 （34-76） years.Among them,97.6% cases （41/42） belonged to Ann Arbor stage Ⅲ/Ⅳ,73.8% （31/42） cases presented with B symptoms,85.7% （36/42） cases had painless lymphadenopathy,52.4% （22/42） cases extranodal involvement,64.3% （27/42） cases elevated lactate dehydrogenase and 45.2% （19/42） cases elevated ββ2-microglobulin at diagnosis.And 40.5% （17/42） cases had 3 points of international prognostic index （IPI） score with the highest proportion.First-line chemotherapy was predominantly CHOP （cyclophosphamide,vincristine,doxorubicin,prednisolone） or CHOP-like-based and complete response was achieved in 44.7% （17/38） of them.The median follow-up time was 40（2-106） months The 1,2,5-year survival rates were 78%,57% and 39% respectively.Statistical analysis showed that IPI was an independent prognostic factor （P =0.009）.Other factors included gender （P =0.311）,age （P =0.263）,with or without B symptoms （P =0.102）,Ki-67 index （P =0.146） as well as the choice of first-line chemotherapy （P =0.292） each had a tendency of affecting; the survival rate,but failed to reach statistical significance.Conclusions Angioimmunoblastic T-cell lymphoma is a major type of peripheral T-cell lymphoma.Most AITL patients are elders with a late stage.The disease generally displays an aggressive clinical course and poor prognosis.