摘要
<正>淋巴管平滑肌瘤病(Lymphangioleiomyomatosis,LAM)是与肺部囊性病变、乳糜积液、腹部肿瘤有关的一种罕见的系统性肿瘤疾病,包括淋巴管平滑肌瘤,血管平滑肌脂肪瘤[1]。LAM的临床表现不具有特异性,临床特点主要有进行性呼吸困难、自发性气胸、胸痛、乳糜胸等。在多于50%的LAM患者可发现自发性或复发性气胸[2]。LAM的肺外表现往往出现肾脏、肝脏、脾脏及腹膜后的肿块和(或)淋巴结肿大、囊性淋巴管瘤等[3]。1 流行
引文
[1] Travis WD,Brambilla E,Nicholson AG,et al.The 2015 World Health Organization Classification of Lung Tumors:Impact of Genetic,Clinical and Radiologic Advances Since the 2004 Classification[J].Journal of Thoracic Oncology,2015,10(9):1243-1260
[2] 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(4):522-5
[3] Sobiecka M.[Lymphangioleiomyomatosis - new concepts on pathogenesis,diagnosis and treatment][J].Wiadomosci Lekarskie,2016,69(2 pt 1):123
[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.An Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guideline[J].Am J Respir Crit Care Med,2017:1337-1348
[5] 陈铭诗,张杨,刘万里,等.男性肺淋巴管肌瘤病长期误诊为慢性支气管炎[J].临床误诊误治,2017,30(10):33-34
[6] 郝兴亮.肺淋巴管平滑肌瘤病一例[J].中华肺部疾病杂志(电子版),2016(4):444-445
[7] Liu F,Lunsford EP,Tong J,et al.Real-Time Monitoring of Tumorigenesis,Dissemination,& Drug Response in a Preclinical Model of Lymphangioleiomyomatosis/Tuberous Sclerosis Complex[J].Plos One,2013,7(6):e38589
[8] 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].国际药学研究杂志,2017,44(2):151-156
[9] 任思颖,胡衍,欧阳若芸,等.结节性硬化症与淋巴管平滑肌瘤病的动物模型研究进展[J].中华结核和呼吸杂志,2016,39(12):977-979
[10] Prizant H,Taya M,Lerman I,et al.Estrogen maintains myometrial tumors in a lymphangioleiomyomatosis model[J].Endocr Relat Cancer,2016,23(4):265
[11] Johnson SR,Cordier JF,Lazor R,et al.European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis[J].European Respiratory Journal,2010,35(1):14-26
[12] Mccormack FX,Gupta N,Finlay GR,et al.Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines:Lymphangioleiomyomatosis Diagnosis and Management[J].American Journal of Respiratory & Critical Care Medicine,2016,194(6):748
[13] 刘杰,钟南山,陈荣昌,等.结节性硬化症相关型和散发型肺淋巴管肌瘤病30例临床回顾研究[J].中华肺部疾病杂志(电子版),2017,10(5):514-520
[14] Xu KF,Zhang P,Tian X,et al.The role of vascular endothelial growth factor-D in diagnosis of lymphangioleiomyomatosis(LAM)[J].Respiratory Medicine,2013,107(2):263-268
[15] 林娟,赖国祥,文文,等.结节硬化症基因与血管内皮生长因子-D对淋巴管肌瘤病的诊治价值[J].中华结核和呼吸杂志,2017,40(9):709-711
[16] 徐文帅.西罗莫司对淋巴管肌瘤病生存率以及脂代谢的影响[D].北京:北京协和医学院,2016
[17] Ando K,Kurihara M,Kataoka H,et al.The efficacy and safety of low-dose sirolimus for treatment of lymphangioleiomyomatosis[J].Respiratory Investigation,2013,51(3):175
[18] 徐文帅,徐凯峰.淋巴管肌瘤病诊疗进展[J].国际呼吸杂志,2016,36(8):620-624
[19] Chang WYC,Cane JL,Maruti K,et al.A 2-year randomised placebo-controlled trial of doxycycline for lymphangioleiomyomatosis[J].European Respiratory Journal,2014,43(4):1114-1123
[20] Lesma E,Chiaramonte E,Ancona S,et al.Anti-EGFR antibody reduces lung nodules by inhibition of EGFR-pathway in a model of lymphangioleiomyomatosis[J].Biomed Res Int,2015,2015:315240