伴危险器官受累的儿童多系统朗格罕斯细胞组织细胞增生症临床分析  

Clinical Analysis of Multi-system Langerhans cell Histiocytosis in Children with Dangerous Organ Involved

作　　者：李丹 赖华 李蕙 史宏 周敏 贺钰磊 金晟娴 高钰 

机构地区：[1]成都市妇女儿童中心医院小儿血液与肿瘤科,四川成都610091

出　　处：《四川医学》2017年第3期283-286,共4页Sichuan Medical Journal

摘　　要：目的探讨累及危险器官的儿童多系统朗格罕斯细胞组织细胞增生症(multi-system langerhans cell histiocytosis,MS-LCH)的临床表现、分组、治疗情况及预后随访。方法对我院2008年至2015年间收治的12例伴危险器官受累的MS-LCH儿童患者进行回顾性分析及随访。结果发病年龄小,临床表现多样化,治疗6周总有效率33%,治疗12个月总有效率75%。随访至2016年6月,早期治疗反应差的高危组有8例,其中复发2例,再化疗后治愈;死亡3例,其中2例为并发Evans综合症的患者。早期治疗反应佳的危险组有4例,其中复发1例,死亡1例,为该复发病例未治死亡。结论伴危险器官受累的MS-LCH儿童患者多数早期治疗反应差;高危组患者有更高的死亡率;高危组和危险组复发率相似,但复发时不伴危险器官受累的患者再化疗预后较好;并发Evans综合征的患者预后差。Objective To investigate the clinical manifestations,grouping,treatment and prognosis of multi-system langer-hans cell histiocytosis（ multi-system langerhans cell histiocytosis,MS-LCH） in children with dangerous organs involved. Method 12 patients with MS-LCH combined with dangerous organs involved who were admitted to our hospital during 2008 and 2015 were retrospectively analyzed and followed up. Results The age of onset was small and the clinical manifestations were diverse. The to-tal effective rate was 33% for 6 weeks of treatment,75% for 12 months of treatment. Up to June 2016,there were 8 cases of poor response to early treatment in high risk group. Among them,2 cases relapsed and cured after re chemotherapy. There were 3 cases of death,of which,2 cases were complicated with Evans syndrome. There were 4 patients with good response to early treatment in risk group. Among them,1 case recurred,1case died,which was the recurrence case died without treatment. Conclusions The ma-jority of children with MS-LCH and dangerous organs involved have poor response to early treatment. Patients in the high-risk group have a higher mortality rate. The recurrence rate of the high-risk group and the risk group are similar,but the rechemotherapyprog-nosis of the patients withoutdangerous organs involved is better. Patients with Evans syndrome have poor prognosis.

关 键 词：朗格罕斯细胞组织细胞增生症 多系统 儿童 治疗 EVANS综合征 

分 类 号：R725.5[医药卫生—儿科]