目的探讨儿童郎格罕细胞组织细胞增生症的临床特点。方法收集某院57例住院患儿资料,分析其临床、病理及免疫组织化学特点。结果平均发病年龄2.34岁,男女发病比例1.48:1。皮肤累及率50.88%。皮疹形态多样,以头面部、躯干部出血性斑丘疹、脂溢性或丘疹样皮疹多见,部分为疱疹、结痂、色素脱失。发热、肝脾大发生率分别为54.39%、56.14%。淋巴结累及率31.58%,以颈部和腹股沟淋巴结为主。骨质浸润发生率24.56%,主要累及颅骨,还可累及锁骨和四肢骨等。结论儿童郎格罕细胞组织细胞增生症可累及多个脏器,表现多样,以年龄分组比较,皮疹在婴幼儿组更多见,其他主要临床表现两组无显著差异。 Objective To investigate the clinical features of Langerhans cell histiocytosis in children. Methods The data of 57 hospitalized children in a hospital were collected and their clinical, pathological and immunohistochemical characteristics were analyzed. Results The average age of onset was 2.34 years, the incidence of male to female ratio was 1.48: 1. Skin involvement rate of 50.88%. Rash forms diverse, with head and face, torso hemorrhagic rash, seborrhea or papular rash more common, part of herpes, crusting, depigmentation. Fever, hepatosplenomegaly rates were 54.39%, 56.14%. The incidence of lymph node involvement was 31.58%, mainly in the neck and inguinal lymph nodes. The incidence of bone infiltration 24.56%, mainly involving the skull, but also involving the clavicle and limb bone and so on. Conclusion Children Langerhans cell histiocytosis can affect multiple organs, showing diversity, age group, rash in infants and young children more common, other major clinical manifestations of two groups no significant difference.