| 龙庆玲,李长钢.儿童朗格汉斯细胞组织细胞增生症的诊治新进展[J].安徽医药,2019,23(6):1065-1068. |
| 儿童朗格汉斯细胞组织细胞增生症的诊治新进展 |
| New progress in diagnosis and treatment of Langerhans cell histiocytosis in children |
| 投稿时间：2018-03-14 |
| DOI： |
| 中文关键词: 朗格汉斯细胞组织细胞增生症 树突状细胞 诊治进展 |
| 英文关键词: Langerhans cell histiocytosis Dendritic cells Progress |
| 基金项目: |
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| 摘要点击次数: 1864 |
| 全文下载次数: 499 |
| 中文摘要: |
| 朗格汉斯细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)是一组以CD1a+、CD207+树突状细胞异常增生为特点的组织细胞增生性疾病,临床表现复杂多样,可侵犯单器官、多器官及多系统,以骨骼、皮肤及垂体损伤常见,分为单系统LCH、高危多系统LCH、低危多系统LCH及特定部位LCH四类；目前主要有炎症性增生学说与肿瘤性增生假说,现多认为与丝裂原活化蛋白激酶(MAPK)通路异常活化密切相关。病理仍是诊断的金标准,而液体活检将可能成为新的无创性确诊手段,根据侵犯的部位、范围及程度制定个体化的治疗方案。近年来,靶向治疗已成为研究的热点,并有望取代传统的治疗方案,但在儿童中的使用仍处于临床研究阶段。现就儿童LCH临床特点及诊治新进展进行综述,以期提高临床诊疗水平。 |
| 英文摘要: |
| Langerhans cell histiocytosis (LCH) is a kind of histiocytic hyperplastic disease,characterized by abnormal proliferation of CD1a+ and CD207+ dendritic cells with heterogeneous clinical features.The special cells can be involved in various organs and systems,frequently in skeleton,skin and pituitary.At present,it can be classified into four types:single system LCH,high risk organ multi-system LCH,low risk organ multi-system LCH and specific LCH.The pathogenesis is not clear,with inflammatory hyperplasia and tumor hyperplasia as the main hypotheses,nevertheless,it is commonly considered to be closely related to excessive activation of mitogen-activated protein kinase (MAPK) signaling pathway now.Pathology is still the golden standard for diagnosis,but liquid biopsy may become a new noninvasive diagnostic method in the future.According to the location,extent and severity of the invasion,individualized therapy could be developed.In recent years,targeted therapy has become a hotspot in research and may replace the conventional therapy,but it is still in clinical research stage for children.In order to improve diagnosis and treatment of this disease on clinical level,this article reviews the clinical characteristics and new progress of diagnosis and treatment of LCH in children. |
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