成年肺朗格汉斯细胞组织细胞增生症一例报道并文献复习  

Adult Pulmonary Langerhans-cell Histiocytosis:a Case Report and Literature Review

作　　者：孙爽[1] 赵世峰[1] SUN Shuang;ZHAO Shifeng(Department of Respiratory Medicine,Chinese PLA General Hospital,Beijing 100000,China)

机构地区：[1]中国人民解放军总医院呼吸内科,北京市100000

出　　处：《实用心脑肺血管病杂志》2020年第6期117-120,共4页Practical Journal of Cardiac Cerebral Pneumal and Vascular Disease

摘　　要：肺朗格汉斯细胞组织细胞增生症(PLCH)是一组临床表现各异的综合征,较为罕见,发病率尚不清楚,但其占成年人弥漫性间质性肺疾病的3%~5%,临床极易被误诊。虽然PLCH并非致命,但延迟诊断或治疗会导致器官功能严重受损,影响生活质量。本文分析1例成年PLCH的临床资料并进行文献复习,从PLCH的临床特征、诊断及治疗提高临床对其认识,避免误诊。Pulmonary Langerhans cell histiocytosis(PLCH)is a group of syndromes with different clinical manifestations.Its rare and incidence was unknown,but it accounts for about 3%to 5%of adult diffuse interstitial lung disease.Clinically easily misdiagnosed.Although PLCH is not fatal,delayed diagnosis or treatment can lead to severe impairment of organ function and reduced quality of life.This paper analyzes the clinical data of an adult PLCH and reviews the literature,improve the clinical understanding of PLCH from clinical characteristics,diagnosis and treatment,so as to avoid misdiagnosis.

关 键 词：郎格尔汉斯细胞组织细胞增多症 肺 成年人 诊断 治疗 病例报告 文献复习 

分 类 号：R551.1[医药卫生—血液循环系统疾病]