朗格罕斯细胞组织细胞增生症初诊临床特征分析  

Analysis of clinical manifestations of 43 children with Langerhans cell histiocytosis at diagnosis

作　　者：沈洁[1] 汤永民[1] 石淑文[1] 宋华[1] 杨世隆[1] 魏健[1] 赵芬英[1] 潘斌华[1] 徐卫群[1] 张玲燕[1] 茅君卿[1] 

机构地区：[1]浙江大学医学院附属儿童医院血液肿瘤科,浙江杭州310003

出　　处：《临床儿科杂志》2009年第8期723-726,共4页Journal of Clinical Pediatrics

摘　　要：目的探讨朗格罕斯细胞组织细胞增生症患儿的临床及预后特点,寻找可能相关的病因学证据。方法回顾性统计分析2003年10月至2009年1月于浙江大学医学院附属儿童医院确诊并住院治疗的43例朗格罕斯细胞组织细胞增生症患儿的临床表现及实验室检查结果,并进行电话随访。结果43例患儿中,<2岁31例(占72.09%);最常见的主诉是体表肿块(22例,占51.16%),高于发热、皮疹及其他主诉(P<0.05);初诊时血液系统受累39例(占90.70%),明显高于其他系统(P<0.01);11例初诊时EBVIgG阳性(25.58%);15例初诊时CRP升高(34.88%),9例外周血淋巴细胞亚群比例异常(20.93%)。结论朗格罕斯细胞组织细胞增生症可以侵犯全身各个含有网状内皮组织的器官,临床表现各异,多发于2岁以下儿童,发病可能与病毒感染、免疫紊乱等有关,详尽的体格检查和实验室检查在诊治中非常重要。Objective To analyze the clinical features of the disease by studying clinical manifestations of 43 children with newly diagnosed Langerhans cell histiocytosis （LCH）. Methods The clinical manifestations and the laboratory data of the 43 LCH children diagnosed and treated in our hospital from October 2003 to January 2009 were collected and analyzed, the follow-up was done via phone calls. Results Thirty-one patients （72.09%） were younger than 2 years old at diagnosis with the most common complaint of superficial mass （51.16%, P 〈 0.05） ; the blood system was the most frequently involved organ seen in 39 （90.70%） patients at the first visit （P 〈 0.01 ）; 11 （25.58%） patients were found to be EBV IgG positive; CRP was elevated in 15 patients （34.88%）; peripheral lymphocyte subpopulation was found to be abnormal in 9 （20.93%） patients. Conclusions Langerhans cell histiocytosis can attack many organs of the human body, including reticuloendothelium tissue, and cause various clinical manifestations. Etiology and pathogenesis of the disease are still unknown. Thorough physical and accessory examination is vital in the diagnostic processure.

关 键 词：朗格罕斯细胞组织细胞增生症 临床表现 实验室检查 预后 

分 类 号：R725.5[医药卫生—儿科]