安徽省淋巴管肌瘤病22例临床分析  

Clinical analysis of 22 cases of Lymphangioleiomyomatosis in Anhui province

作　　者：孙宛君 高梦茹 胡代菊 刘梅 韦炜 倪伟建 徐飞[3] 胡晓文 SUN Wan-jun;GAO Meng-ru;HU Dai-ju;LIU Mei;WEI Wei;NI Wei-jian;XU Fei;HU Xiao-wen(Provincial Hospital Affiliated to Anhui Medical University,Hefei,Anhui 230032,China;Department of Gastroenterology,the Sixth People′s Hospital,Fuyang,Anhui 236000,China;Department of Pulmonary and Critical Care Medicine,the First Affiliated Hospital of USTC,Hefei,Anhui 230001,China;Department of Pulmonary Function,the First Affiliated Hospital of USTC,Hefei,Anhui 230001,China;Department of Radiology,the First Affiliated Hospital of USTC,Hefei,Anhui 230001,China;Department of Pharmacy,the First Affiliated Hospital of USTC,Hefei,Anhui 230001,China)

机构地区：[1]安徽医科大学附属省立医院,安徽合肥230032 [2]安徽省阜阳市第六人民医院消化科,安徽阜阳236000 [3]中国科学技术大学附属第一医院(安徽省立医院)呼吸与危重症医学科,安徽合肥230001 [4]中国科学技术大学附属第一医院(安徽省立医院)肺功能室,安徽合肥230001 [5]中国科学技术大学附属第一医院(安徽省立医院)医学影像科,安徽合肥230001 [6]中国科学技术大学附属第一医院(安徽省立医院)药剂科,安徽合肥230001

出　　处：《临床肺科杂志》2021年第4期487-492,共6页Journal of Clinical Pulmonary Medicine

基　　金：国家重点研发计划(No.2016YFC0901502)。

摘　　要：目的总结安徽省淋巴管肌瘤病(LAM)患者临床特征,提高诊治水平。方法回顾分析2009年1月1日至2019年12月31日中科大附属第一医院LAM患者临床资料。结果本研究纳入22例女性LAM患者,平均诊断年龄(37±11)岁。近5年LAM诊断病例较前增加超三倍。所有患者胸部高分辨CT显示肺弥漫薄壁囊性病变。长期误诊20例(91%),平均误诊58月,误诊诊断为多发性肺大泡9例(41%)、肾癌6例(27%)和慢阻肺6例(23%)。最终病理确诊11例,临床诊断11例,其中VEGF-D≥800ng/L协助临床诊断5例(23%),TSC 2例,肾AML 2例,乳糜胸1例和肺外淋巴管肌瘤1例。临床表现以呼吸困难17例(77%)最常见,肺外症状10例(45%),并发气胸9例,复发率67%。西罗莫司有效率88%,气胸患者胸膜固定术后复发率25%。结论近5年LAM诊断数显著增加。育龄期女性出现呼吸困难、反复气胸,甚至肺外症状,应行胸部高分辨CT寻找特征性囊性病变,完善血清VEGF-D协助诊断LAM。西罗莫司能有效改善病情,并发气胸尽早行胸膜固定术,减少复发。Objective To summarize the clinical characteristics of patients with lymphangioleiomyomatosis(LAM)in Anhui Province,in order to improve the diagnosis and management.Methods The clinical data of patients with LAM in the First Affiliated Hospital of University of Science and Technology of China from January 1,2009 to December 31,2019 were retrospectively analyzed.Results A total of 22 female patients with LAM were enrolled in this cohort,at an average diagnosis age of(37±11)years.The number of LAM diagnosed cases had more than tripled in the past 5 years.High-resolution chest CT showed diffuse thin-walled cystic lesions involving bilateral lungs in all patients.91%of the patients had been misdiagnosed for average 58 months,and common misdiagnoses included 9 cases of multiple pulmonary bullae(41%),6 cases of kidney cancer(27%)and 6 cases of chronic obstructive pulmonary disease(23%).Finally,11 patients were diagnosed by biopsy,others were clinically diagnosed including 5 cases based on VEGF-D≥800 ng/L,2 cases of TSC,2 cases of renal AML,1 case of chylothorax and 1 case of lymphangioleiomyoma.The most common clinical manifestation was dyspnea in 17 cases(77%),followed by 10 cases(45%)of extrapulmonary symptoms,9 cases of complicated pneumothorax,and a recurrence rate of 67%.Sirolimus was effective in 88%of the patients,and the recurrence rate of pneumothorax after pleural fixation was 25%.Conclusion The number of LAM diagnosed cases has increased significantly in the past 5 years.Women of childbearing age presenting with dyspnea,recurrent pneumothorax or even extrapulmonary symptoms should timely perform high-resolution chest CT and serum VEGF-D to make a right diagnosis.Sirolimus effectively improves the condition of most patients with LAM.Patients with pneumothorax should be performed pleurodesis as soon as possible to avoid recurrence.

关 键 词：淋巴管肌瘤病 高分辨率CT 血清血管内皮生长因子-D 

分 类 号：R734.2[医药卫生—肿瘤]