朗格汉斯细胞组织细胞增生症的研究  

Study on Langerhans Cell Histiocytosis

作　　者：赵巍 何云燕[2] ZHAO Wei;HE Yun-yan(Department of Pediatrics,Liuzhou Worker's Hospital,Liuzhou 545003,Guangxi,China;Department of Pediatrics,the First Affiliated Hospital of Guangxi Medical University,Nanning 530021,Guangxi,China)

机构地区：[1]广西柳州市工人医院儿科,广西柳州545003 [2]广西医科大学第一附属医院儿科,广西南宁530021

出　　处：《医学信息》2022年第13期57-61,共5页Journal of Medical Information

摘　　要：朗格汉斯细胞组织细胞增多症(LCH)由骨髓前体细胞异常克隆扩增引起,可导致多器官受累和功能障碍,因而临床表现多样化。LCH发病机制目前仍不清楚,研究提示其可能与克隆增殖、免疫失调、遗传因素等因素有关。LCH的治疗目标是降低死亡率、预防再激活和减少后遗症。临床上多根据受累部位而合理分型,采用分层治疗的策略。单系统LCH通常采用保守的局部治疗为主、预后较好,多系统LCH治疗主要为化疗、靶向治疗等,其低风险组预后较好,而高风险组预后较差。此外,需要警惕LCH治疗后远期的后遗症。本文现就LCH的流行病学、病理特点与诊断、临床表现与分型、发病机制、治疗与预后等研究作一综述。Langerhans cell histiocytosis(LCH)is caused by abnormal clonal expansion of bone marrow progenitor cells,which can lead to multiple organ involvement and dysfunction,and thus the clinical manifestations are diverse.The pathogenesis of LCH is still unclear,and studies suggest that it may be related to cloning proliferation,immune disorders,genetic factors and other factors.The goal of LCH treatment is to reduce mortality,prevent reactivation and reduce sequelae.In clinical practice,it is usually classified reasonably according to the affected parts,and the strategy of stratified treatment is adopted.Single-system LCH is usually treated with conservative local therapy,and the prognosis is good.Multi-system LCH is mainly treated with chemotherapy and targeted therapy.The prognosis of low risk group is better,and the prognosis of high risk group is poor.In addition,it is necessary to be vigilant about the long-term sequelae after LCH treatment.This article reviews the epidemiology,pathological features and diagnosis,clinical manifestations and classification,pathogenesis,treatment and prognosis of LCH.

关 键 词：朗格汉斯细胞组织细胞增多症 朗格汉斯细胞 骨髓前体细胞 

分 类 号：R733[医药卫生—肿瘤]