朗格汉斯细胞组织细胞增多症126例临床分析

Langerhans cell histiocytosis： clinical analysis of 126 cases

目的分析朗格汉斯细胞组织细胞增多症患儿的临床特点。方法对2006--2011年我院126例朗格汉斯细胞组织细胞增多症进行临床回顾性分析。结果126例患者中年龄最小为2个月，最大9岁，男女比例是2．5：1。皮肤病变呈湿疹样、脂溢性、出血性斑丘疹样、黄色结节样和白色斑疹样。在3种传统临床分型中，Letterer—Siwe病所占比例最高，患者年龄最小，受累器官最多，多伴有肝脾肿大、x线胸片异常、造血功能损伤、多部位骨受累，临床分级主要是Ⅲ级和Ⅳ级。Hand—Schfiller-Christian病的患儿临床分级各级均有分布，以Ⅱ级最多。骨嗜酸细胞肉芽肿患者年龄最大，除骨骼以外未发现其他器官受累，临床分级大部分为I级。治疗采用外科手术或联合化疗。结论朗格汉斯细胞组织细胞增多症皮损表现有特征性，临床表现多样，病理有诊断意义，治疗和疗效取决于疾病程度和受累器官。

Objective To analyze lesional and clinical characteristics of Langerbans 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 LetterSiwe disease were associated with hepatosplenomegaly, abnormal chest X-ray, impaired hematopoietic function and multifocal hone 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 withⅡ 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.