儿童多系统朗格汉斯细胞组织细胞增生症8例临床分析

发布时间：2018-02-28 03:53

  本文关键词： 朗格汉斯细胞组织细胞增生症 儿童 多系统　出处：《吉林大学》2017年硕士论文　论文类型：学位论文

【摘要】：朗格汉斯细胞组织细胞增生症是病理性朗格汉斯细胞(pathologic Langerhans Cells,p LCs)增生并在多种器官中聚集所形成的疾病。在儿童中为少见病,本病的发病机制至今仍不明确。因受累组织器官的不同而导致临床症状、体征差异性较大;同时在临床转归上也差异显著,故该病的诊治对临床医师存在很多困惑。目的:探讨儿童多系统受累LCH的临床特征、诊治及预后特点,旨在提高儿科等相关医生对本病的认识及诊疗水平。方法:收集吉林大学第一医院小儿肿瘤科2013年2月至2015年11月经病理确诊并接受规范化综合治疗的8例多系统受累的LCH患儿的完整临床资料,并结合相关文献进展,归纳总结多系统受累的儿童LCH的临床表现、治疗反应、不良反应及转归特征。结果:我科治疗的8例多系统受累的LCH患儿中,男性6例(6/8,75%),女性2例(2/8,25%),平均年龄为25个月。其中新生儿2例(2/8,25%),婴幼儿5例(5/8,62.5%)。主要临床表现为骨骼受累(7/8,87.5%)、肺部受累(6/8,75%)、皮疹(5/8,62.5%)、肝功异常(3/8,37.5%)、耳受累(3/8,37.5%)、淋巴结受累(3/8,37.5%)、尿崩(1/8,12.5%)。病初治疗反应率为100%,治疗相关毒性主要表现为Ⅱ°-Ⅲ°的骨髓抑制和Ⅰ°-Ⅱ°的肝功能异常,且尤其表现在婴幼儿中。年长儿化疗相关毒性不明显,恶心、呕吐等消化道症状较轻微。但治疗过程中病情进展4例(4/8,50%);随诊期疾病再激活2例(2/7,28.6%),无事件生存率为71.4%(5/7)。总生存率为7/8(87.5%)。平均随访时间为13.3个月。对治疗过程中疾病进展及随诊期间疾病再激活的病例,经采用含有依托泊苷的加强治疗后,仍表现良好治疗反应。结论:在本组观察病例中,年幼儿发病率较高;多系统LCH患儿对化疗反应良好,预后尚可;婴幼儿在治疗过程中易出现病情的反复或进展;对病情进展或再激活病例,经加强治疗后,仍可表现较好的治疗反应。
[Abstract]:Langerhans cell histiocytosis is a disease of pathological Langerhans cell pathologic Langerhans Cellsp LCs. it is a rare disease in children. The pathogenesis of the disease is still unclear. Because of the different tissues and organs involved, the clinical symptoms and signs are quite different. At the same time, there are significant differences in the clinical outcome. Therefore, the diagnosis and treatment of the disease has a lot of confusion for clinicians. Objective: to explore the clinical characteristics, diagnosis, treatment and prognosis of LCH in children with multiple system involvement. The aim of this study was to improve the knowledge, diagnosis and treatment of paediatrics and other related doctors. Methods: from February 2013 to 2015, the department of pediatric oncology in the first hospital of Jilin university was collected from 8 patients who were diagnosed pathologically and accepted standardized comprehensive treatment from February 2013 to 2015. Complete clinical data of children with LCH, The clinical manifestations, therapeutic reactions, adverse reactions and outcome characteristics of LCH in children with multi-system involvement were summarized and summarized. Results: among the 8 children with multi-system involvement of LCH treated in our department, the clinical manifestations, therapeutic reactions, adverse reactions and outcome characteristics were summarized. Six men (6 / 8 / 75), two women (2 / 8) have a mean age of 25 months, of which 2 are newborns (2 / 8) and 5 / 8 / 862.5 are involved. The main clinical manifestations are skeletal involvement 78-87.5m, lung involvement 6- / 87.75, rashes 5- / 885 / 62.5, liver dysfunction 3- / 87.5m, ear involvement 3r837.5m, ear involvement 3r837.5m, lymph node breakdown 38.8rpm. The initial therapeutic response rate of the disease was 100. The related toxicity of treatment was mainly myelosuppression of 鈪，

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