29例儿童颅骨郎格汉斯细胞组织细胞增生症临床特点分析  

Clinical analysis of 29 children with skull cranial langerhans cell histiocytosis

作　　者：董蔚蔚 李苗苗 陈鹏宇 李新星[1] Dong Weiwei;Li Miaomiao;Chen Pengyu;Li Xinxing(Department of Neurosurgery,Shengjing Hospital Affiliated to China Medical University,Shenyang 110004,China)

机构地区：[1]中国医科大学附属盛京医院神经外科,沈阳110004

出　　处：《中国医师进修杂志》2019年第2期131-134,共4页Chinese Journal of Postgraduates of Medicine

基　　金：辽宁省重点研发计划指导计划项目(2018225094).

摘　　要：目的探讨儿童颅骨郎格汉斯细胞组织细胞增生症的临床特点,并提高临床认识及治疗效果。方法回顾性分析2012年1月至2018年1月就诊于中国医科大学附属盛京医院治疗的29例颅骨郎格汉斯细胞组织细胞增生症患儿临床资料。结果颅骨骨质破坏均以溶骨性骨破坏为主,额骨病变5例,颞骨病变10例,枕骨病变2例,颅骨多灶性病变12例;合并脊柱椎体骨质受累10例,周身多骨受累5例;合并尿崩4例;皮疹3例,肝脾肿大2例,淋巴结肿大1例。随访0.5~5.0年,手术联合化疗组18例,其中单系统受累患儿9例病情消退,2例病情平稳,1例患儿病情进展;多系统受累1例病情消退,2例病情平稳,3例病情进展,多系统受累患儿预后相对不佳(P<0.05);单纯手术组11例,其中单系统受累5例病情消退,1例病情平稳,1例病情进展;多系统受累1例病情平稳,3例病情进展。结论儿童颅骨郎格汉斯细胞组织细胞增生症表现单发或多灶性颅骨溶骨性病损改变,常伴有周身多系统多器官受累且多系统受累患儿预后不佳。Objective To investigate the clinical characteristics of skull cranial langerhans cell histiocytosis in children and improve clinical understanding and therapeutic effect Methods A retrospective clinical study was conducted in 29 cases of skull cranial langerhans cell histiocytosis treated in Shengjing Hospital Affiliated to China Medical University from January 2012 to January 2018.Results The main lesion of skull bone was dissolved bone. Among them,there were 5 cases of frontal bone lesions,10 cases of temporal bone lesions,2 cases of occipital lesions,and 12 cases of multiple lesions in the skull;There were 10 cases of vertebral lesions and 5 cases of multiple bone lesions involved around the body. Other clinical manifestations included insipidus in 4 cases,erythra in 3 cases,hepatosplenomegaly in 2 cases and lymphadenectasis in 1 case. All cases were followed up for 0.5 to 5.0 years. In the group of combined surgery and chemotherapy treatment,the prognosis of single system involvement cases was that 9 patients were cured,2 patients were stable and 1 patients were progressing,while the prognosis of multiple systems involvement cases was that 1 patient was cured,2 patients were stable and 3 patients were progressing. The prognosis of patient with multiple systems lesions was relatively poor compared to that of the cases of single system lesion and there was statistical difference. In the pure operation group,the prognosis of single system involvement cases was that 5 patients were cured,1 patient was stable and 1 patient was progressing,while the prognosis of multiple systems involvement cases was that 1 patient was stable and 3 patients were progressing.Conclusions Children with skull cranial langerhans cell histiocytosis present single or multiple cranial osteolytic lesions. It is often accompanied by multiple systems lesions around the body,and prognosis of children with multiple systems lesions was is relatively poor.

关 键 词：郎格汉斯细胞组织细胞增生症 儿童 颅骨 预后 

分 类 号：R725.9[医药卫生—儿科]