本文關(guān)鍵詞： 朗格漢斯細(xì)胞組織細(xì)胞增生癥 兒童 多系統(tǒng)　出處：《吉林大學(xué)》2017年碩士論文　論文類型：學(xué)位論文

【摘要】：朗格漢斯細(xì)胞組織細(xì)胞增生癥是病理性朗格漢斯細(xì)胞(pathologic Langerhans Cells,p LCs)增生并在多種器官中聚集所形成的疾病。在兒童中為少見病,本病的發(fā)病機(jī)制至今仍不明確。因受累組織器官的不同而導(dǎo)致臨床癥狀、體征差異性較大;同時(shí)在臨床轉(zhuǎn)歸上也差異顯著,故該病的診治對臨床醫(yī)師存在很多困惑。目的:探討兒童多系統(tǒng)受累LCH的臨床特征、診治及預(yù)后特點(diǎn),旨在提高兒科等相關(guān)醫(yī)生對本病的認(rèn)識(shí)及診療水平。方法:收集吉林大學(xué)第一醫(yī)院小兒腫瘤科2013年2月至2015年11月經(jīng)病理確診并接受規(guī)范化綜合治療的8例多系統(tǒng)受累的LCH患兒的完整臨床資料,并結(jié)合相關(guān)文獻(xiàn)進(jìn)展,歸納總結(jié)多系統(tǒng)受累的兒童LCH的臨床表現(xiàn)、治療反應(yīng)、不良反應(yīng)及轉(zhuǎn)歸特征。結(jié)果:我科治療的8例多系統(tǒng)受累的LCH患兒中,男性6例(6/8,75%),女性2例(2/8,25%),平均年齡為25個(gè)月。其中新生兒2例(2/8,25%),嬰幼兒5例(5/8,62.5%)。主要臨床表現(xiàn)為骨骼受累(7/8,87.5%)、肺部受累(6/8,75%)、皮疹(5/8,62.5%)、肝功異常(3/8,37.5%)、耳受累(3/8,37.5%)、淋巴結(jié)受累(3/8,37.5%)、尿崩(1/8,12.5%)。病初治療反應(yīng)率為100%,治療相關(guān)毒性主要表現(xiàn)為Ⅱ°-Ⅲ°的骨髓抑制和Ⅰ°-Ⅱ°的肝功能異常,且尤其表現(xiàn)在嬰幼兒中。年長兒化療相關(guān)毒性不明顯,惡心、嘔吐等消化道癥狀較輕微。但治療過程中病情進(jìn)展4例(4/8,50%);隨診期疾病再激活2例(2/7,28.6%),無事件生存率為71.4%(5/7)�？偵媛蕿�7/8(87.5%)。平均隨訪時(shí)間為13.3個(gè)月。對治療過程中疾病進(jìn)展及隨診期間疾病再激活的病例,經(jīng)采用含有依托泊苷的加強(qiáng)治療后,仍表現(xiàn)良好治療反應(yīng)。結(jié)論:在本組觀察病例中,年幼兒發(fā)病率較高;多系統(tǒng)LCH患兒對化療反應(yīng)良好,預(yù)后尚可;嬰幼兒在治療過程中易出現(xiàn)病情的反復(fù)或進(jìn)展;對病情進(jìn)展或再激活病例,經(jīng)加強(qiáng)治療后,仍可表現(xiàn)較好的治療反應(yīng)。
[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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