以多发软组织包块为主要表现的老年朗格汉斯细胞组织细胞增生症一例  

A Case of Senile Langerhans Cell Histiocytosis with Soft Tissue Mass as the Main Manifestation

作　　者：邢明泉 吴维霞 孙晓星[1] 王其凯 韩浩 葛洪峰[1] XING Mingquan;WU Weixia;SUN Xiaoxing;WANG Qikai;HAN Hao;GE Hongfeng(Department of Hematology,the People's Hospital of Bozhou City in Anhui Province,Bozhou 236800,China;Image Center,the People's Hospital of Bozhou City in Anhui Province,Bozhou 236800,China)

机构地区：[1]安徽省亳州市人民医院血液内科,亳州236800 [2]安徽省亳州市人民医院影像中心,亳州236800

出　　处：《罕见病研究》2023年第3期432-435,共4页Journal of Rare Diseases

摘　　要：朗格汉斯细胞组织细胞增生症(LCH)是一组以朗格汉斯细胞增殖并破坏局部组织为特点的罕见病。LCH发病群体主要为儿童,老年人发病率极低,相关研究较少。LCH病变可累及包括骨组织、淋巴结、皮肤、肝脏、脾脏等在内的多处器官和系统,但以累及全身多处软组织并以软组织包块为主要表现的老年LCH患者实属罕见。本文报道1例以多发软组织包块为主要表现的老年LCH临床特点、治疗及预后,以期为临床提供参考。Langerhans cell histiocytosis(LCH)is a rare disease characterized by the proliferation of Langerhans cells and the destruction of local tissue.LCH large occurs in children,whilst incidence of the elderly population is extremely low,and there are few related studies.LCH lesions can involve multiple organs and systems,including bone tissue,lymph nodes,skin,liver,and spleen.However,it is rare that multiple soft tissues are implicated for eldly patients with LCH and present with soft tissue mass as the main manifestation.Here is a report on the clinical features,treatment and prognosis of an elderly LCH with multiple soft tissue masses as the main manifestation,in order to provide clinical reference.

关 键 词：朗格汉斯细胞组织细胞增生症 软组织包块 罕见病 

分 类 号：R739.5[医药卫生—肿瘤]