关键词: 组织细胞增生症，郎格尔汉斯细胞, 回顾性研究

Abstract: SHU Ye, ZENG Ying-hong, ZHOU Bin, CHEN Wei-jian, TANG Jian-ping. Department of Dermatology, Hunan Children's Hospital, Changsha 410007, China Corresponding author: TANG Jian-ping, Email：jpingtang@126.com 【Abstract】 Objective To analyze lesional and clinical characteristics of Langerhans cell histiocytosis in children. Methods A clinical retrospective study was performed on 126 patients with Langerhans cell histiocytosis collected from 2006 to 2011 at the Hunan Children's Hospital. Results Of the 126 patients, the youngest was 2 months old, and the oldest was 9 years old. The ratio of male to female was 2.5 ∶ 1. Clinical manifestations included eczematid, seborrheic lesions, hemorrhagic maculopapules, yellow nodules and white macules. Of the three clinical phenotypes of Langerhans cell histiocytosis, Letter-Siwe disease was the most prevalent, and most cases of Letter-Siwe disease were associated with hepatosplenomegaly, abnormal chest X-ray, impaired hematopoietic function and multifocal bone injuries. The clinical grade was mainly III and Ⅳ in patients with Letter-Siwe disease, I in patients with eosinophilic granuloma, and varied from I to Ⅳ in patients with Hand-Schuller-Christian disease with II as the most common. Of these patients, those with eosinophilic granuloma had the oldest average age with bone as the only affected organ, while those with Letter-Siwe disease had the youngest average age with the greatest number of affected organs. The treatment of Langerhans cell histiocytosis included surgical operation and combined chemotherapy. Conclusions Langerhans cell histiocytosis has characteristic skin lesions and diverse clinical manifestations. Pathology has diagnostic significance to Langerhans cell histiocytosis. Therapy strategies and curative effects are dependent on the severity of, and the organs affected by Langerhans cell histiocytosis.

Key words: Retrospective studies