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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 To analyze the efficacy and prognostic factors of modified LAL-HX83 / 90 regimen in children with Langerhans cell histocytosis (LCH). Methods A retrospective analysis of 34 cases of pediatric LCH with pediatric prophylaxis and follow-up data from July 2003 to December 2014 in the First Affiliated Hospital of Armed Police Corps, China with modified DAL-HX83 / 90 protocol was performed. Kaplan-Meier method was used to calculate the overall survival (OS) and event-free survival (EFS). Log-rank test and multivariate COX regression model were used to analyze the influencing factors of EFS. Results (1) The effective rate of induction therapy was 82.4% (28/34). The effective rate of initial treatment after chemotherapy was 88.2% (30/34). (2) All children were followed up for 48 months (range, 13 to 155 months). The 1, 3, 5-year OS was 100.0%, 97.1%, 93.7% The 5-year EFS were 82.4%, 70.3% and 70.3% respectively. The overall recurrence rate was 26.5% (9/34) and the median time to recurrence was 8 months (1-72 months). (3) Univariate analysis showed that multiple system involvement (χ ~ 2 = 10.213, P = 0.001) and induction therapy at 6 weeks were not effective (χ ~ 2 = 9.744, P = 0.002) Analysis showed that multiple system involvement was an independent risk factor for EFS in children with LCH [relative risk (RR) = 9.933, P = 0.037]. Conclusion The modified DAL-HX83 / 90 regimen has better curative effect, less adverse reactions and tolerability for LCH children with impaired single organ system function, but the recurrence rate is still high for patients with impaired multi-organ organ function. Room for improvement. Multiple system involvement is an independent risk factor for EFS in children with LCH.