婴幼儿朗格汉斯细胞组织细胞增生症临床特点和生存分析

Analysis of Manifestations and Survival Rate of Langerhans Cell Hisitocytosis in Infancy

目的探讨婴幼儿朗格汉斯细胞组织细胞增生症患儿的临床特点及生存情况。方法回顾性分析2005年5月至2009年12月初次明确诊断的婴幼儿朗格汉斯细胞组织细胞增生症患儿的临床表现及生存情况。结果共明确诊断25例朗格汉斯细胞组织细胞增生症,中位年龄10个月。多数为多器官功能受累,分级以Ⅲ级或Ⅳ级为主。临床表现多见皮疹、发热、体表包块、黄疸、外耳流脓为主要表现。器官受累依次为皮肤、骨骼、肝脏、肺脏、骨髓等。病理以S-100,CD_1a阳性为主要表现。死亡4例,其中治疗相关死亡1例、呼吸衰竭1例、肝功能衰竭1例、缓解后发生AML-M3合并DIC 1例。结论婴幼儿朗格汉斯细胞组织细胞增生症发生率男性略高于女性。发病多以多器官、功能受累为主要表现。通过皮肤活检获得病理诊断。多数为高危组患者。死亡原因主要由于重要脏器(肺、肝脏)功能受累有关。

Objective To describe the morphology characteristics of skin lesions and clinical course of 25 infant patients with Langerhans cell histioeytosis（LCH）. Methods A retrospective review of the medical records of patients with Langerhans cell histiocytosis in infancy in capital institute of pediatrics from May 2005 to Dee 2009. Results LCH in infancy was characterized by proliferation of Langerhans cell with involvement of skin, hone, lung, liver, bone marrow and other organs. 25 infant patients were muhisystem cases. Immunostaining with S100 and CD1 a was positive in 22 cases. Four patients were died, of whom one died of treatment, one died of lung function, one died of respiratory dysfunction, and one was diagnosed as AML-M3 1 years and a half later, and died of DIC eventually. Conclusions The prevalence of LCH is slightly higher in males. Age of less than 2 years at diagnosis significantly decreases the chances of long-term survival because patients in this age group are more likely to have disseminated disease. It can be diagnosed by skin biopsy. Patients with organ dysfunction tend to have higher morhidities and mortality.