10例成人肺朗格罕斯细胞组织细胞增生症患者临床特征分析及文献复习  

Clinical feature analysis of 10 adult patients with pulmonary langerhans cell histiocytosis and literature review

作　　者：张瑞[1] 刘杰 陈如冲 刘福荣[3] 黄会粉 李道明[4] Zhang Rui;Liu Jie;Chen Ruchong;Liu Furong;Huang Huifen;Li Daoming(Department of Respiratory and Critical Care Medicine,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China;Department of Respiratory and Critical Care Medicine,Guangzhou Institute of Respiratory Health,the First Affiliated Hospital of Guangzhou Medical University,Guangzhou 510120,China;Department of Medical Records,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China;Department of Pathology,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]郑州大学第一附属医院呼吸与危重症医学科,郑州450052 [2]广州呼吸健康研究院广州医科大学第一附属医院呼吸与危重症医学科,广州510120 [3]郑州大学第一附属医院病案科,郑州450052 [4]郑州大学第一附属医院病理科,郑州450052

出　　处：《国际呼吸杂志》2022年第8期589-595,共7页International Journal of Respiration

基　　金：河南省医学科技攻关计划项目(LHGJ20190226)。

摘　　要：目的探讨成人肺朗格罕斯细胞组织细胞增生症(PLCH)的临床特征。方法回顾性分析2011年11月至2020年1月在郑州大学第一附属医院就诊的经肺组织病理确诊为成人PLCH患者的临床资料,总结其症状、影像特点、肺功能和治疗转归,并复习相关文献。结果PLCH患者10例,包括男7例和女3例,中位发病年龄33岁,主要症状为咳嗽(8/10)和胸闷(4/10),CT表现包括结节影(4/10)、囊腔影(4/10)和团块影(1/10)。7例完成肺功能检查,其中2例肺通气功能和弥散功能均正常,2例小气道功能障碍,1例阻塞性通气功能障碍和弥散功能下降,2例混合型通气功能障碍和弥散功能下降。1例患者放弃治疗,9例患者戒一手烟和避免二手烟暴露,其中4例给予化疗和糖皮质激素治疗,5例单纯使用口服泼尼松片治疗。平均随访3年,失访1例,7例病情改善,1例病情恶化。同时,复习24篇文献,总结805例成人PLCH患者的临床资料。结论成人PLCH发病高峰在28~43岁,症状无特异,影像表现有一定特点,但亦可出现罕见团块影,肺通气功能和弥散功能受损时提示肺部病变严重。治疗上戒烟联合糖皮质激素和(或)化疗可改善多数PLCH患者的预后和延缓疾病进展。Objective To investigate the clinical feature of adult patients with pulmonary langerhans cell histiocytosis(PLCH).Methods The clinical data of adult patients with PLCH diagnosed by lung histopathology in the First Affiliated Hospital of Zhengzhou University from November 2011 to January 2020 were retrospectively analyzed,and the clinical symptoms,imaging feature,lung function,and treatment outcome were summarized.Meanwhile,the related literature was reviewed.Results There were 10 patients with PLCH including 7 males and 3 females.The median age of onset was 33 years old.The main symptoms were cough(8/10)and chest tightness(4/10).CT images included nodules(4/10),cystic cavity(4/10),and mass(1/10).Pulmonary function tests were performed in 7 cases,among which 2 cases had normal pulmonary ventilation and normal diffusion function,2 cases had small airway dysfunction,1 case had obstructive ventilation dysfunction and diffusion dysfunction,and 2 cases had mixed ventilation dysfunction and diffusion dysfunction.One patient gave up treatment.Nine patients quit smoking and avoided second-hand smoke exposure,of which 4 patients were treated with chemotherapy and glucocorticoids,and 5 patients were treated with oral prednisone tablets alone.After an average of 3-year follow-up,1 case was lost to follow-up,7 cases improved,and 1 case deteriorated.Meanwhile,24 literatures were reviewed,and the clinical data of 805 cases of adult PLCH were summarized.Conclusions The peak incidence of adult PLCH is between the ages of 28 and 43 years.The symptoms are not specific,and the imaging manifestations have certain characteristics,but rare mass shadow could also appear.When the pulmonary ventilation function and diffusion function are impaired,it indicates severe lung lesions.Therapeutically,smoking cessation in combination with glucocorticoids and/or chemotherapy could improve prognosis and delay disease progression in most patients with PLCH.

关 键 词：肺朗格罕斯细胞组织细胞增生症 成人 临床特征 影像特点 治疗转归 

分 类 号：R563[医药卫生—呼吸系统]