头颈部受累的儿童朗格罕细胞组织细胞增生症34例临床分析

Clinical analysis of 34 cases of Langerhans cell histiocytosis in children with head and neck involvement

目的探讨头颈部受累的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。方法选取34例头颈部受累的LCH患儿,分析其临床特征、临床疗效和预后情况,并依据国际组织细胞协会制定的临床分型标准以及Lavin和Osband临床分级标准进行临床分型和分级,比较不同分型和不同发病年龄LCH患儿的5年无进展生存率。结果34例LCH患儿诱导治疗6周总有效率为94.1%,治疗52周的总有效率为97.1%,随访至2019年4月30日,15例患儿在治疗过程中出现原发病灶消退较慢,1例患儿进展,调整治疗方案后治疗效果较好,5年总生存率率为100%,5年疾病无进展生存率为(53.6±8.9)%。Ⅰ型患儿的5年无进展生存率为100%,Ⅱ型患儿的5年无进展生存率为(83.3±10.8)%,将Ⅰ及Ⅱ型患儿作为一组,其5年无进展生存率为(84.5±5.8)%,高于Ⅲ型患儿的(34.5±10.8)%,差异有统计学意义(P﹤0.05)。依据发病年龄将34例患儿分为≤2岁组(n=12)和﹥2岁组(n=22),结果显示,≤2岁组患儿的5年无进展生存率为(24.9±7.6)%,低于﹥2岁组患儿的(72.1±9.1)%,差异有统计学意义(P﹤0.05)。结论头颈部受累的LCH患儿出现器官功能异常的治疗难度较高,病灶消退较其他部位慢;在治疗期间,需定期进行影像学检查,结合病灶的变化及时调整治疗方案,可以提高无进展生存率,远期预后较好。

Objective To investigate thet clinical features,reatment and prognosis of Langerhans cell histiocytosis (LCH) in children with head and neck involvement.Method A retrospective analysis was performed for the clinical data of 34 cases children with LCH involving head and neck to analyze the clinical features,clinical efficacy and prognosis, besides,the diseases were classified as per the clinical classification criteria established by international histology and cytology society and Lavin-Osband criteria for further comparison regarding the 5-year progression-free survival (PFS) rate of children patients with varying subtypes and age of onset.Result The 34 children of LCH had an overall response rate (ORR) after 6 weeks of induction therapy at 94.1%,and at 52 weeks was 97.1%.Followed-up was finished on April 30, 2019,and there were 15 cases with slow regression of primary lesions in the treatment process,and 1 case had disease progression,which was well controlled after therapy adjustment;in this cohort of patients,the 5- year overall survival (OS) rate was 100% and 5-year PFS rate was (53.6±8.9)%.The 5-year PFS rate in patients with type I disease was 100%, and (83.3±10.8)% for those with type II disease,additionally,it was (84.5±5.8)% in type I and II patients as a pooled group,which was higher than the (34.5±10.8)% among patients with type III disease,showing statistically significant differences (P<0.05).According to the age of onset,34 children were divided into ≤2 years old group (n=12) and >2 years old group (n=22).The results showed that the 5-year PFS rate of children ≤2 years old group (24.9±7.6)%,which was lower compared to the (72.1±9.1)% in children aged >2 years old group (P<0.05).Conclusion It is a challenge to administer appropriate treatment for the children of LCH with head and neck involvement who develop organ dysfunction,the lesion subsides more slowly than the ones in other sites.In the course of treatment,regular review of imaging examination and timely individualized adjustment of treatment based on changes in the lesion can improve the PFS rate and provide acceptable long-term prognosis.