以胆管炎为主要表现的朗格罕细胞组织细胞增多症临床特点分析

目的:总结朗格罕细胞组织细胞增生症(langerhans cell histiocytosis,LCH)的主要临床特点、诊断、治疗以及预后,尤其是以胆管炎为主要表现的病例.方法:回顾性分析2012年7月-2016年4月至湖南省儿童医院确诊并住院治疗的12例LCH患儿的临床表现、实验室检测、骨髓细胞学检查以及治疗情况,治疗以化疗为主.单器官受累8例,多器官系统受累4例.结果:12例LCH患儿中,11例有程度不一的黄疸,8例超声表现为肝大,2例骨骼系统有破坏,6例有肺部侵犯.结论:LCH的诊断是复杂的,可能涉及多个器官系统,其临床表现和疾病变化的过程是从早期一个孤立的表现到后来多系统表现,通过对这些病例的研究可能有助于我们对LCH的病理生理学的认识和理解,从而提高临床对LCH的诊断和治疗.

Analysis of clinical features in cholangitis as the main manifestation of langerhans cell histiocytosis

Objective:To summarize the clinical characteristics of Langerhans cell organization cell hyperplasia disease (LCH),especially the cholangitis is the main performance of the cases.Methods:The clinical manifestations,laboratory examination,bone marrow cytology of 12 patients were retrospectively analyzed,and the relevant literature was reviewed.8 patients had single system disease and the Other 6 with multisystem disease.Results:In 12 children with LCH,11 had a different degree of jaundice.8 cases were performed by ultrasound,and 2 cases had failure of skeletal system.6 cases had lung invasion.Conclusion:The diagnosis of LCH is complex and it may involve multiple organ systems,the clinical manifestations and the changes in the process,from an early stage to an isolated performance to later multiple system performance.