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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 investigate the clinical and prognostic features of children with Langerhans cell histiocytosis in order to find out the possible etiological evidence. Methods The clinical manifestations and laboratory findings of 43 children with Langerhans cell histiocytosis confirmed and hospitalized at Zhejiang Children’s Hospital of Zhejiang University from October 2003 to January 2009 were retrospectively analyzed. Follow up by phone. Results Of the 43 cases, 31 cases (72.09%) were younger than 2 years old. The most common complaint was body surface mass (22 cases, 51.16%), higher than those of fever, rash and other complaints (P <0.05) 39 cases (90.70%) had hematologic involvement and were significantly higher than other systems (P <0.01). Eleven patients had EBV Ig positive (25.58%) at the time of first visit, 15 (34.88%) newly diagnosed and 9 peripheral blood Abnormal lymphocyte subsets (20.93%). Conclusion Langerhans cell histiocytosis can invade the body with various reticuloendothelial organs, different clinical manifestations, mainly in children under 2 years of age, the incidence may be related to viral infections, immune disorders and other detailed physical examination and Laboratory examination in the diagnosis and treatment is very important.