改良DAL-HX83/90方案治疗儿童朗格汉斯细胞组织细胞增生症的疗效及预后因素  

Efficacy and prognostic factors of langerhans cell histocytosis in children treated with modified DAL-HX83/90 protocol

作　　者：周云[1] 苗丽霞[1] 李彦珊[1] 孙岩峰[1] 王军[1] 赵扬[1] 金德奎 张向兰[1] 刘秋玲[1] ZHOU Yun;MIAO Lixia;LI Yanshan;SUN Yanfeng;WANG Jun;ZHAO Yang;JIN Dekui;ZHANG Xianglan;LIU Qiuling(Department of Pediatrics, General Hospital of Chinese People's Armed Police Force, Beijing 100039, China)

机构地区：[1]武警总医院儿科,北京100039

出　　处：《中华灾害救援医学》2017年第4期198-202,共5页Chinese Journal of Disaster Medicine

摘　　要：目的分析改良DAL-HX83/90方案治疗儿童朗格汉斯细胞组织细胞增生症(langerhans cell histocytosis,LCH)的疗效及预后因素。方法回顾性分析武警某三甲医院儿科2003-07至2014-12采用改良DAL-HX83/90方案初治的34例LCH患儿的病例及随访资料。应用Kaplan-Meier方法计算其总体生存率(overall survival,OS)和无事件生存率(event free survival,EFS),采用Log-rank检验及多因素COX回归模型分析EFS的影响因素。结果 (1)诱导治疗有效率为82.4%(28/34),化疗结束初治有效率为88.2%(30/34)。(2)所有患儿均获得有效随访,中位随访时间48个月(13~155个月);患儿1、3、5年OS分别为100.0%、97.1%、93.7%,1、3、5年EFS分别为82.4%、70.3%、70.3%。全组复发率为26.5%(9/34),中位复发时间8个月(1~72个月)。(3)单因素分析显示,多系统受累(χ~2=10.213,P=0.001)和6周诱导治疗无效(χ~2=9.744,P=0.002)是影响LCH患儿EFS的因素;多因素分析表明,多系统受累是影响LCH患儿EFS的独立危险因素[相对危险度(relative risk,RR)=9.933,P=0.037]。结论改良DAL-HX83/90方案对于单系统、无脏器功能受损LCH患儿疗效较好,不良反应少,可耐受,但对于多系统脏器功能受损者复发率仍较高,疗效有待提高。多系统受累是影响LCH患儿EFS的独立危险因素。Objective This study objective was to analyse the efficacy and prognostic factors of modified DAL-HX83/90protocol in treatment of langerhans cell histocytosis(LCH)in children.Methods A retrospective analysis was conducted on the clinicalrecords and follow-up data of34children with LCH treated with modified DAL-HX83/90protocol in a third-grade class-A hospital of PAPfrom July2003to December2014.The Kaplan-Meier estimator was applied to calculate the overall survival(OS)and event free survival(EFS),log-rank test and multivariate Cox proportion hazard regression model were used for the analysis of the factors impacting on EFS.Results(1)The effective rate of inductive treatment was82.4%(28/34),the effective rate of the initial treatment after chemotherapy was88.2%(30/34).(2)All the children with LCH were followed up effectively,the median follow-up time was48months(13~155months);amongthe34children with LCH,the1-year OS,3-year OS and5-year OS were100.0%,97.1%and93.7%and1-year EFS,3-year EFS and5-year EFS were82.4%,70.3%and70.3%,respectively.The recurrence rate was26.5%(9/34),the median time to relapse was8months(1~72months).(3)The univariate analysis showed that multisystem involvement(χ2=10.213,P=0.002)and ineffective inductive treatment(χ2=10.213,P=0.001)were the risk factors of EFS in children with LCH.The multivariate analysis revealed that multisystem involvementwas the independent risk factor of EFS in children with LCH(RR=9.933,P=0.037).Conclusions The modified DAL-HX83/90protocolis an effective and tolerable therapy for LCH children without organ dysfunction and has less adverse reactions,while its curative effect onpatients with multiple organ dysfunction needs to be improved.Multisystem involvement is the independent risk factor of EFS in childrenwith LCH.

关 键 词：改良DAL-HX83/90方案 朗格汉斯细胞组织细胞增生症 预后 

分 类 号：R725.5[医药卫生—儿科]