肺淋巴管平滑肌瘤病临床特点及诊疗现状

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Abstract 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 with effective treatment.Conclusion PLAM is a multi-organ disease.PLAM occurs commonly in child-bearing age women,the most common manifestation is progressive dyspnea,the imaging and pathological manifestation is characteristic.Compared with invasive diagnosis methods such as bronchoscopy,thoracoscopy,and open-thoracic lung biopsy,VEGF-D will be more widely used for diagnosis and assessment of disease in future work.At present,targeted therapy and lung transplantation are effective treatment methods.

Key words： Pulmonary lymphangioleiomyomatosis Vascular endothelial growth factor-D Misdiagnosis Status of diagnosis and treatment

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	LIU Jia
	LI Yu-rong

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LIU Jia,LI Yu-rong. Clinical features and current status of diagnosis and treatment of pulmonary lymphangioleiomyomatosis[J]. 中国当代医药, 2020, 27(35): 64-68.

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https://www.dangdaiyiyao.com/EN/ OR https://www.dangdaiyiyao.com/EN/Y2020/V27/I35/64

[1]	刘杰，钟南山，陈荣昌，等.淋巴管肌瘤病并肾血管平滑肌脂肪瘤41例临床回顾研究[J].国际呼吸杂志，2017，37（22）：1720-1726.
[2]	Cornog JL Jr，Enterline HT.Lymphangiomyoma，a benign lesion of chyliferous lymphatics synonymous with lymphangiopericytoma[J].Cancer，1966，19（12）：1909-1930.
[3]	刘亦庸，张熙曾，魏鉴，等.一种罕见疾病-胸内淋巴管肌瘤病1例[J].中国肿瘤临床，1986，13：116-118.
[4]	Gupta N，Finlay GA，Kotloff RM，et al.Lymphangioleiomyomatosis Diagnosis and Management：High-Resolution Chest Computed Tomography，Transbronchial Lung Biopsy，and Pleural Disease Management[J].Am J Resp Critcare Med，2017，196（10）：1337-1348.
[5]	纵单单，欧阳若芸.雷帕霉素成功治疗肺及腹膜后淋巴管肌瘤病1例报道及文献复习[J].中南大学学报（医学版），2012，37（9）：963-967.
[6]	胡代菊，胡晓文，汪萤，等.西罗莫司治疗肺淋巴管肌瘤病伴巨大肾血管平滑肌脂肪瘤一例[J].中华结核和呼吸杂志，2019，42（1）：62-64.
[7]	张龙举，梁毅，钟小宁，等.国内三十余年肺淋巴管肌瘤病130例临床与病理文献复习分析[J].中国全科医学，2015，18（3）：329-334.
[8]	叶书高，陈员，郑明峰，等.肺移植治疗肺淋巴管肌瘤病9例临床分析[J].中华胸心血管外科杂志，2019，35（1）：6-9.
[9]	凌公豪，彭碧荣，周军，等.肺淋巴管肌瘤病CT表现[J].实用放射学杂志，2018，34（4）：522-525.
[10]	周莉，欧阳若芸，刘婷.21例肺淋巴管平滑肌瘤病临床诊治与随访回顾性分析[J].中国现代医学杂志，2019，29（23）：58-64.
[11]	崔晗，田欣伦，徐文帅.经支气管镜肺活检对肺淋巴管肌瘤病的诊断价值[J].国际呼吸杂志，2015，35（10）：771-774.
[12]	李燕，梁颖，李英明.肺外淋巴管平滑肌瘤病3例临床病理观察[J].诊断病理学杂，2018，25（8）：549-555.
[13]	牟艳，黄嘉楠，叶伶.血清血管内皮生长因子-D（VEGF-D）在多发囊泡性肺疾病（PLD）鉴别诊断中的价值[J].复旦学报，2019，46（1）：47-51.
[14]	吴喜端，曾庆思，黄绥丹，等.HRCT 扫描对肺淋巴管平滑肌瘤病的肺部结构研究[J].广州医科大学学报，2017，45（5）：5-8.
[15]	Johnosn SR，Whale CI，Hubbard RB，et al.Survival and Disease progression in UK patients with lymphangioleiomyomatosis[J].Thorax，2004，59（9）：800-803.
[16]	赵天明，倪吉祥，胡学华.淋巴管平滑肌瘤病最新研究进展[J].中国全科医学，2017，20（24）：3056-3062.
[17]	刘芳，平芬.淋巴管平滑肌瘤病的研究进展[J].华北理工大学学报（医学版），2019，21（4）：325-329.
[18]	王坚，叶伶，牟艳.雌激素在肺淋巴管肌瘤病发病机制中的研究进展[J].国际呼吸杂志，2014，34（22）：1737-1741.
[19]	Hohman DW，Noghrehkar D，Ratnayake S.Lymphangioleiomyomatosis：a review[J].Eur J Intern Med，2008，19：319-324.
[20]	李树娇，周敏，张利，等.肺淋巴管肌瘤病CT 特点及与血清学指标的关系[J].中国医学计算机成像杂志，2018，24（4）：311-315.
[21]	田欣伦，王俊，徐凯峰.淋巴管肌瘤病：从分子研究到靶向治疗[J].国际药学研究杂，2017，44（2）：151-156.
[22]	徐凯峰.西罗莫司治疗淋巴管肌瘤病专家共识（2018）[J].中华结核和呼吸杂志，2019，42（2）：92-97.