摘要
目的探讨儿童朗格罕细胞组织细胞增生症（LCH）的临床特征、治疗及预后因素。方法对首都医科大学附属北京儿童医院2007年1月至2012年12月收治的217例初发LCH患儿进行回顾性分析。患儿分为多器官高危险组（I组）、多器官低危险组（Ⅱ组）和单器官受累组（Ⅲ组），给予相应强度化疗。应用SPSS17．0统计学软件进行组间差异比较，分析受累器官、早期治疗反应与预后的相关性。结果217例患儿包括男127例，女90例，发病比例为1．4：1．0，中位发病年龄为36个月（2个月-14岁）。I组132例（60．8％），中位发病年龄为20个月；Ⅱ组33例（15．3％），中位发病年龄为42个月；m组52例（23．9％），中位发病年龄为72个月。受累部位以骨最常见，为176例（81．2％）。217例患儿中复发55例（25．3％），死亡12例。预计3年总生存率为90．78％，3年无事件生存率为76．5％。化疗并发症以骨髓抑制、肝功能损害、感染为主，分别占48．4％（105例）、24．0％（52例）、12．4％（27例）。危险器官受累及早期治疗反应（6周）是预后不良的相关因素（X。=10．60、12．84，P=0．017、0．001）。结论儿童LCH男童发病比例略高于女童，发病高峰年龄为1～3岁。骨骼是最常见的受累部位。危险器官受累及早期治疗反应是影响预后的关键因素，需及早进入补救治疗。
Objective To investigate the clinical characteristics, treatment, and prognostic factors for Langer- hans cell histiocytosis （LCH） in children. Methods A retrospective review of patients diagnosed as LCH was carried out between January 2007 and December 2012 in Beijing Children＇s Hospital, Capital Medical University. Target patients were divided into multi - organ high - risk groups （ Group I ）, multi - organ low - risk groups （ Group II ）, single - or- gan groups （Groupm） , and the corresponding intensity of chemotherapy was given. SPSS 17.0 statistical software was used to analyze the findings. The correlations among the affected organ, the early treatment response and the prognosis were analyzed. Results A total of 217 patients were analyzed including 127 boys and 90 girls with ratio of 1.4 ： 1.0 and a median age of 36 months （ ranged from 2 months to 14 years） on the diagnosis of LCH, and there were 132 cases （60.8%） in group ,33 cases （ 15.3% ） in group Ⅱ and 52 cases （23.9%） in group m. The median age on diagno- sis was 20 months in group I ,42 months in group Ⅱ and 72 months in group Ⅲ. The most frequently affected organ was the bone （ 176 cases,81.2% ）. Among 217 patients,55 cases （25.3%） had recurrence and 12 cases died. The rate of 3 - year overall survival was expected to be 90.78%. The rate of 3 - year event free survival was expected to be 76.5%. Myelosuppression,liver function damage and infection were the most common side effects due to chemotherapy with the percentages of 48.4% （105 cases） ,24.0% （52 cases） and 12.4% （27 cases）. Risk organs involvement and no - response to initial therapy （ after 6 weeks） indicated a worse prognosis （X2 = 10.60,12.84, P = O. 017,0. 001 ）. Conclusions Incidence of LCH in boys is slightly higher than girls in children. Peak age at onset of LCH in children is 1 - 3 years old. Bone is the most frequent involved organ. Involvement of risk organs and no - response to initial therapy are key factors i
出处
《中华实用儿科临床杂志》
CSCD
北大核心
2017年第15期1141-1144,共4页
Chinese Journal of Applied Clinical Pediatrics
