小儿朗格汉斯细胞组织细胞增生症24例临床分析  

Clinical analysis of 24 cases of Langerhans cell histiocytosis in children

作　　者：薛宁 潘凯丽[2] 张静静[2] 

机构地区：[1]西北妇女儿童医院,陕西西安710000 [2]第四军医大学西京医院儿科,陕西西安710032

出　　处：《中国妇幼健康研究》2015年第6期1298-1301,共4页Chinese Journal of Woman and Child Health Research

摘　　要：目的探讨小儿朗格汉斯细胞组织细胞增生症（LCH）的临床特点、分型分级、治疗及预后，提高临床对小儿LCH的认识。方法对西京医院2008年10月至2014年10月收治的24例朗格汉斯细胞组织细胞增生症患儿的临床表现、实验室检查结果及诊疗情况进行回顾性分析。结果24例患儿的发病年龄为3个月-12岁，男16例，女8例。主要临床表现为发热、出皮疹、骨骼受累、肝脾大、淋巴结肿大、突眼、尿崩症、外耳道溢脓及其他脏器改变。不同分型组在脏器受累范围和程度指标上有明显不同。I型I级5例，Ⅱ型I级4例．Ⅱ型Ⅱ级2例，Ⅲ型Ⅱ级3例，Ⅲ型Ⅲ级2例，Ⅲ型Ⅳ级8例。7例经过手术加化疗后治愈，9例单部位骨骼受累采用手术刮除后痊愈，3例行化疗方案，目前病情稳定，1例复发，1例死亡，1例遗留尿崩症，2例失访。结论小儿朗格汉斯细胞组织细胞增生症临床表现多种多样，诊断时需结合临床表现、实验室检查、影像学检查，确诊依靠组织病理学检查。根据分型与分级的不同，给予不同的治疗方案。预后与受累器官数目与功能受损情况密切相关。Objective To explore the clinical features, classification and grading, therapy and prognosis of childhood Langerhans cell histiocytosis （LCH） so as to improve the awareness of LCH in clinics. Methods Clinical data of 24 cases suffering from LCH were retrospectively analyzed in Xijing Hospital of Fourth Military Medical University treated during the period of October 2008 to October 2014. The clinical presentations, laboratory examinations, diagnosis and treatment were discussed. Results The onset age of 24 cases ranged 3 months to 12 years old, including 16 males and 8 females. The main clinical manifestations were fever, rash, hepatosplenomegaly, lymphadenopathy, exophthalmos, diabetes insipidus, external auditory canal overflow pus, skeletal and other organs involvement. Different type groups were significantly different in the scope and extent of organ involvement. Among 24 patients, there were 5 cases of type I stage Ⅰ , 4 cases of type Ⅱ stage Ⅰ, 2 cases of type Ⅱ stage Ⅱ , 3 cases of type Ⅲ stage Ⅱ, 2 cases of type m stage Ⅲ, and 8 cases of type m stage IV. Seven cases were cured by surgical curettage combined with chemotherapy, and 9 cases of single-site bone involvement were cured after surgical curettage. Three cases accepted routine chemotherapy and currently were in stable condition. One case recurred, one died, one case had the sequelae of diabetes insipidus, and two cases were lost in follow-up. Conclusion Due to the variety of clinical manitestations of LCH in children, the diagnosis of LCH should be based on clinical manifestations, laboratory tests, features of imaging and histopathology. Different treatments should be given according to different types and grades of the disease. Prognosis is closely related to the number of organs involved and functional damage.

关 键 词：儿童 朗格汉斯细胞 组织细胞增生症 临床分析 

分 类 号：R551.1[医药卫生—血液循环系统疾病]