肺淋巴管平滑肌瘤病临床特点及诊疗现状  

Clinical features and current status of diagnosis and treatment of pulmonary lymphangioleiomyomatosis

作　　者：刘佳 李玉荣[1] LIU Jia;LI Yu-rong(Geriatrics Center,the Fourth People's Hospital of Shenyang City,Liaoning Province,Shenyang 110000,China)

机构地区：[1]沈阳市第四人民医院老年医学中心,辽宁沈阳110000

出　　处：《中国当代医药》2020年第35期64-68,共5页China Modern Medicine

摘　　要：目的总结我国近30年肺淋巴管肌瘤病(PLAM)的文献资料,分析PLAM的临床与病理特征、治疗进展及预后。方法通过万方、维普中文数据库和中国生物医学文献数据库,检索1986年1月~2020年4月国内有关PLAM的文献资料进行汇总分析。结果最终纳入文献150篇,共289例患者,其中女283例(97.9%),男6例(2.1%);最常见临床表现为呼吸困难(90例,31.1%),其次为气促、咳嗽、胸闷、咯血;最常见的肺外表现或合并症为气胸(78例,66.1%);典型胸部影像学表现为双肺弥漫性多发大小不等的薄壁囊状影(278例,96.2%),其次为气胸(64例,23.0%)和胸腔积液(49例,17.6%);243例提到了确诊方式,最主要的确诊方式为病理确诊(241例,99.2%),其次为典型的肺HRCT表现同时伴有血清血管内皮细胞生长因子-D(VEGF-D)增高(2例,0.8%);病理检查结果可见肺组织结构破坏,肺泡扩张并相互融合形成肺大泡,肺间质中异常平滑肌细胞增生,以梭形、上皮样为主,围绕淋巴管、血管分布;文献中提及免疫组化结果的病例为158例,最常见的阳性结果为人类黑色素瘤单克隆抗体-45(HMB-45)(123例,77.8%),其次为平滑肌肌动蛋白(SMA)(118例,74.7%);157例患者提到初步诊断,误诊率为63.1%,误诊最多的为肺间质纤维化(26例,16.6%);治疗方面,靶向治疗逐渐增多,且效果显著,肺移植治疗18例,治疗均有效。结论PLAM为可以累及多器官的疾病,主要发生于育龄期女性,呼吸困难是最常见症状,并且有特征性影像学和病理学表现,相比于气管镜、胸腔镜、开胸肺活检等有创确诊方式,VEGF-D在未来工作中将更加广泛用于诊断及评估病情,目前靶向治疗及肺移植是有效的治疗手段。Objective To summarize the document literature of pulmonary lymphangioleiomyomatosis(PLAM)in Chinese population in the last thirty years,and to analyze the clinical and pathological features,treatment progress and prognosis of PLAM.Methods Through Wanfang,Weipu Chinese database and China Biology Medicine database(CBM),the document literature of PLAM from January 1986 to April 2020 were retrieved and analyzed.Results A total of 150 articles were included in this study,among 289 patients,283 cases(97.9%)were females and 6 cases(2.1%)were males.Dyspnea was the most common clinical manifestation(90 cases,31.1%),followed by shortness of breath,cough,chest tightness and haemoptysis.The most common extrapulmonary manifestation or complication was pneumothorax(78 cases,66.1%).The most common chest imaging manifestation was multiple different sized thin-walled cystic shadows in whole lung(278 cases,96.2%),followed by pneumothorax(64 cases,23.0%)and pleural effusion(49 cases,17.6%).There were 243 cases mentioned the diagnostic method.The diagnosis was confirmed by pathology(241 cases,99.2%),followed by typical HRCT findings with increased vascular endothelial growth factor-D(VEGF-D)(2 cases,0.8%).Pathological features showed that the lung tissue was destroyed,the alveoli expand and fuse with each other to form bullae,and the abnormal smooth muscle cell proliferation in the lung interstitium,mainly fusiform and epithelioid,distributed around lymphatic vessels and blood vessels.There were 158 cases with immunohistochemical results mentioned in the literature.The most common positive result was human melanoma monoclonal antibody-45(HMB-45)(123 cases,77.8%),followed by smooth muscle actin(SMA)(118 cases).There were 157 patients mentioned the initial diagnosis,the misdiagnosis rate was 63.1%,and the most misdiagnosed was pulmonary interstitial fibrosis(26 cases,16.6%).In terms of treatment,targeted therapies have gradually increased,and the effects have been remarkable.There were 18 cases of lung transplantation were treated wi

关 键 词：肺淋巴管肌瘤病 血管内皮细胞生长因子-D 误诊 诊疗现状 

分 类 号：R563.9[医药卫生—呼吸系统]