淋巴管平滑肌瘤病治療的研究進展_Chinese Journal of Respiratory and Critical Care Medicine

Authors：

成小亮 ,  楊小東

四川大學華西醫院呼吸與危重癥醫學科（四川成都 610041）;

Corresponding?author：

楊小東, Email: yang-xiaodong@163.com

Keywords：

DOI：

10.7507/1671-6205.202205016

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Citation： 成小亮, 楊小東. 淋巴管平滑肌瘤病治療的研究進展. Chinese Journal of Respiratory and Critical Care Medicine, 2023, 22(8): 600-604. doi: 10.7507/1671-6205.202205016 Copy

1.	McCormack FX, Travis WD, Colby TV, et al. Lymphangioleiomyomatosis: calling it what it is: a low-grade, destructive, metastasizing neoplasm. Am J Respir Crit Care Med, 2012, 186(12): 1210-1212.
2.	McCormack FX, Gupta N, Finlay GR, et al. Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management. Am J Respir Crit Care Med, 2016, 194(6): 748-761.
3.	Steagall WK, Pacheco-Rodriguez G, Darling T N, et al. The lymphangioleiomyomatosis lung cell and its human cell models. Am J Respir Cell Mol Biol, 2018, 58(6): 678-683.
4.	Johnson SR, Cordier JF, Lazor R, et al. European Respiratory Society Guidelines for the Diagnosis and Management of Lymphangioleiomyomatosis. Eur Respir J, 2010, 35(1): 14-26.
5.	Taveira-DaSilva AM, Moss J. Clinical features, epidemiology, and therapy of lymphangioleiomyomatosis. Clin Epidemiol, 2015, 7: 249-257.
6.	Goncharova EA, Goncharov DA, Eszterhas A, et al. Tuberin regulates p70 S6 kinase activation and ribosomal protein S6 phosphorylation. A role for the TSC2 tumor suppressor gene in pulmonary lymphangioleiomyomatosis (LAM). J Biol Chem, 2002, 277(34): 30958-30967.
7.	Taveira-DaSilva AM, Hathaway O, Stylianou M, et al. Changes in lung function and chylous effusions in patients with lymphangioleiomyomatosis treated with sirolimus. Ann Intern Med, 2011, 154(12): 797-805, 292-293.
8.	McCormack FX, Inoue Y, Moss J, et al. Efficacy and safety of sirolimus in lymphangioleiomyomatosis. N Engl J Med, 2011, 364(17): 1595-1606.
9.	Bissler J J, McCormack FX, Young LR, et al. Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis. N Engl J Med, 2008, 358(2): 140-151.
10.	Young L, Lee HS, Inoue Y, et al. Serum VEGF-D a concentration as a biomarker of lymphangioleiomyomatosis severity and treatment response: a prospective analysis of the Multicenter International Lymphangioleiomyomatosis Efficacy of Sirolimus (MILES) trial. Lancet Respir Med, 2013, 1(6): 445-452.
11.	Taveira-DaSilva AM, Jones AM, Julien-Williams P, et al. Long-term effect of sirolimus on serum vascular endothelial growth factor D levels in patients with lymphangioleiomyomatosis. Chest, 2018, 153(1): 124-132.
12.	Bissler J J, Kingswood J C, Radzikowska E, et al. Everolimus for angiomyolipoma associated with tuberous sclerosis complex or sporadic lymphangioleiomyomatosis (EXIST-2): a multicentre, randomised, double-blind, placebo-controlled trial. Lancet, 2013, 381(9869): 817-824.
13.	Darling TN. Topical sirolimus to treat tuberous sclerosis complex (TSC). JAMA Dermatol, 2018, 154(7): 761-762.
14.	Wataya-Kaneda M, Ohno Y, Fujita Y, et al. Sirolimus gel treatment vs placebo for facial angiofibromas in patients with tuberous sclerosis complex: a randomized clinical trial. JAMA Dermatol, 2018, 154(7): 781-788.
15.	中華醫學會呼吸病學分會間質性肺疾病學組, 淋巴管肌瘤病共識專家組, 中國醫學科學院罕見病研究中心, 等. 西羅莫司治療淋巴管肌瘤病專家共識(2018). 中華結核和呼吸雜志, 2019, 42(2): 92-97.
16.	Ando K, Kurihara M, Kataoka H, et al. Efficacy and safety of low-dose sirolimus for treatment of lymphangioleiomyomatosis. Respir Investig, 2013, 51(3): 175-183.
17.	Bee J, Fuller S, Miller S, et al. Lung function response and side effects to rapamycin for lymphangioleiomyomatosis: a prospective national cohort study. Thorax, 2018, 73(4): 369-375.
18.	Yoon HY, Hwang JJ, Kim DS, et al. Efficacy and safety of low-dose Sirolimus in Lymphangioleiomyomatosis. Orphanet J Rare Dis, 2018, 13(1): 204.
19.	Xu KF, Tian X, Yang Y, et al. Rapamycin for lymphangioleiomyomatosis: optimal timing and optimal dosage. Thorax, 2018, 73(4): 308-310.
20.	Gupta N, Lee H, Young LR, et al. Analysis of the MILES cohort reveals determinants of disease progression and treatment response in lymphangioleiomyomatosis. Eur Respir J, 2019, 53(4): 1802066.
21.	Goncharova EA, Goncharov DA, Li H, et al. mTORC2 is required for proliferation and survival of TSC2-null cells. Mol Cell Biol, 2011, 31(12): 2484-2498.
22.	Goncharova EA, Goncharov DA, Fehrenbach M, et al. Prevention of alveolar destruction and airspace enlargement in a mouse model of pulmonary lymphangioleiomyomatosis (LAM). Sci Transl Med, 2012, 4(154): 154ra134.
23.	Krymskaya VP, Courtwright AM, Fleck V, et al. A phase II clinical trial of the Safety Of Simvastatin (SOS) in patients with pulmonary lymphangioleiomyomatosis and with tuberous sclerosis complex. Respir Med, 2020, 163: 105898.
24.	Le K, Steagall WK, Stylianou M, et al. Effect of beta-agonists on LAM progression and treatment. Proc Natl Acad Sci U S A, 2018, 115(5): E944-E953.
25.	Lu C, Lee HS, Pappas GP, et al. A phase II clinical trial of an aromatase inhibitor for postmenopausal women with lymphangioleiomyomatosis. Ann Am Thorac Soc, 2017, 14(6): 919-928.
26.	Babaei-Jadidi R, Dongre A, Miller S, et al. Mast cell tryptase release contributes to disease progression in lymphangioleiomyomatosis. Am J Respir Crit Care Med, 2021.
27.	Liu HJ, Lizotte PH, Du H, et al. TSC2-deficient tumors have evidence of T cell exhaustion and respond to anti-PD-1/anti-CTLA-4 immunotherapy. JCI Insight, 2018, 3(8).
28.	Maisel K, Merrilees MJ, Atochina-Vasserman E N, et al. Immune checkpoint ligand PD-L1 is upregulated in pulmonary lymphangioleiomyomatosis. Am J Respir Cell Mol Biol, 2018, 59(6): 723-732.
29.	Bendeck MP, Conte M, Zhang M, et al. Doxycycline modulates smooth muscle cell growth, migration, and matrix remodeling after arterial injury. Am J Pathol, 2002, 160(3): 1089-1095.
30.	Matsui K, Takeda K, Yu ZX, et al. Role for activation of matrix metalloproteinases in the pathogenesis of pulmonary lymphangioleiomyomatosis. Arch Pathol Lab Med, 2000, 124(2): 267-275.
31.	Odajima N, Betsuyaku T, Nasuhara Y, et al. Matrix metalloproteinases in blood from patients with LAM. Respir Med, 2009, 103(1): 124-129.
32.	Moses M A, Harper J, Folkman J. Doxycycline treatment for lymphangioleiomyomatosis with urinary monitoring for MMPs. N Engl J Med, 2006, 354(24): 2621-2622.
33.	Baldi B G, Ribeiro C C. Doxycycline in lymphangioleiomyomatosis: not all questions are answered. Eur Respir J, 2014, 43(5): 1536-1537.
34.	Chang WY, Cane JL, Kumaran M, et al. A 2-year randomised placebo-controlled trial of doxycycline for lymphangioleiomyomatosis. Eur Respir J, 2014, 43(4): 1114-1123.
35.	El-Chemaly S, Taveira-Dasilva A, Goldberg HJ, et al. Sirolimus and autophagy inhibition in lymphangioleiomyomatosis: results of a phase I clinical trial. Chest, 2017, 151(6): 1302-1310.
36.	El-Chemaly S, Taveira-DaSilva A, Bagwe S, et al. Celecoxib in lymphangioleiomyomatosis: results of a phase I clinical trial. Eur Respir J, 2020, 55(5).
37.	Almoosa KF, Ryu JH, Mendez J, et al. Management of pneumothorax in lymphangioleiomyomatosis: effects on recurrence and lung transplantation complications. Chest, 2006, 129(5): 1274-1281.
38.	Hayashida M, Seyama K, Inoue Y, et al. The epidemiology of lymphangioleiomyomatosis in Japan: a nationwide cross-sectional study of presenting features and prognostic factors. Respirology, 2007, 12(4): 523-530.
39.	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. Am J Respir Crit Care Med, 2017, 196(10): 1337-1348.
40.	Kurihara M, Mizobuchi T, Kataoka H, et al. A total pleural covering for lymphangioleiomyomatosis prevents pneumothorax recurrence. PLoS One, 2016, 11(9): e163637.
41.	Suzuki E, Kurihara M, Tsuboshima K, et al. The effects of total pleural covering on pneumothorax recurrence and pulmonary function in lymphangioleiomyomatosis patients without history of pleurodesis or thoracic surgeries for pneumothorax. J Thorac Dis, 2021, 13(1): 113-124.
42.	Gonano C, Pasquier J, Daccord C, et al. Air travel and incidence of pneumothorax in lymphangioleiomyomatosis. Orphanet J Rare Dis, 2018, 13(1): 222.
43.	Taveira-DaSilva AM, Moss J. Epidemiology, pathogenesis and diagnosis of lymphangioleiomyomatosis. Expert Opin Orphan Drugs, 2016, 4(4): 369-378.
44.	Hecimovic A, Jakopovic M, Pavlisa G, et al. Successful treatment of pulmonary and lymphatic manifestations of lymphangioleiomyomatosis with sirolimus. Lymphology, 2015, 48(2): 97-102.
45.	Itkin M, McCormack FX. Nonmalignant Adult Thoracic Lymphatic Disorders. Clin Chest Med, 2016, 37(3): 409-420.
46.	Taveira-DaSilva AM, Johnson SR, Julien-Williams P, et al. Pregnancy in lymphangioleiomyomatosis: clinical and lung function outcomes in two national cohorts. Thorax, 2020, 75(10): 904-907.
47.	Shen L, Xu W, Gao J, et al. Pregnancy after the diagnosis of lymphangioleiomyomatosis (LAM). Orphanet J Rare Dis, 2021, 16(1): 133.
48.	Xu KF, Xu W, Liu S, et al. Lymphangioleiomyomatosis. Semin Respir Crit Care Med, 2020, 41(2): 256-268.
49.	Ando K, Okada Y, Akiba M, et al. Lung transplantation for lymphangioleiomyomatosis in Japan. PloS one, 2016, 11(1): e146749.
50.	Khawar MU, Yazdani D, Zhu Z, et al. Clinical outcomes and survival following lung transplantation in patients with lymphangioleiomyomatosis. J Heart Lung Transplant, 2019, 38(9): 949-955.
51.	Salman J, Ius F, Sommer W, et al. Long-term results of bilateral lung transplantation in patients with end-stage pulmonary lymphangioleiomyomatosis. Prog Transplant, 2019, 29(2): 115-121.
52.	Groetzner J, Kur F, Spelsberg F, et al. Airway anastomosis complications in de novo lung transplantation with sirolimus-based immunosuppression. J Heart Lung Transplant, 2004, 23(5): 632-638.
53.	King-Biggs MB, Dunitz JM, Park SJ, et al. Airway anastomotic dehiscence associated with use of sirolimus immediately after lung transplantation. Transplantation, 2003, 75(9): 1437-1443.
54.	El-Chemaly S, Goldberg HJ, Glanville A R. Should mammalian target of rapamycin inhibitors be stopped in women with lymphangioleiomyomatosis awaiting lung transplantation? Expert Rev Respir Med, 2014, 8(6): 657-660.

1. McCormack FX, Travis WD, Colby TV, et al. Lymphangioleiomyomatosis: calling it what it is: a low-grade, destructive, metastasizing neoplasm. Am J Respir Crit Care Med, 2012, 186(12): 1210-1212.
2. McCormack FX, Gupta N, Finlay GR, et al. Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management. Am J Respir Crit Care Med, 2016, 194(6): 748-761.
3. Steagall WK, Pacheco-Rodriguez G, Darling T N, et al. The lymphangioleiomyomatosis lung cell and its human cell models. Am J Respir Cell Mol Biol, 2018, 58(6): 678-683.
4. Johnson SR, Cordier JF, Lazor R, et al. European Respiratory Society Guidelines for the Diagnosis and Management of Lymphangioleiomyomatosis. Eur Respir J, 2010, 35(1): 14-26.
5. Taveira-DaSilva AM, Moss J. Clinical features, epidemiology, and therapy of lymphangioleiomyomatosis. Clin Epidemiol, 2015, 7: 249-257.
6. Goncharova EA, Goncharov DA, Eszterhas A, et al. Tuberin regulates p70 S6 kinase activation and ribosomal protein S6 phosphorylation. A role for the TSC2 tumor suppressor gene in pulmonary lymphangioleiomyomatosis (LAM). J Biol Chem, 2002, 277(34): 30958-30967.
7. Taveira-DaSilva AM, Hathaway O, Stylianou M, et al. Changes in lung function and chylous effusions in patients with lymphangioleiomyomatosis treated with sirolimus. Ann Intern Med, 2011, 154(12): 797-805, 292-293.
8. McCormack FX, Inoue Y, Moss J, et al. Efficacy and safety of sirolimus in lymphangioleiomyomatosis. N Engl J Med, 2011, 364(17): 1595-1606.
9. Bissler J J, McCormack FX, Young LR, et al. Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis. N Engl J Med, 2008, 358(2): 140-151.
10. Young L, Lee HS, Inoue Y, et al. Serum VEGF-D a concentration as a biomarker of lymphangioleiomyomatosis severity and treatment response: a prospective analysis of the Multicenter International Lymphangioleiomyomatosis Efficacy of Sirolimus (MILES) trial. Lancet Respir Med, 2013, 1(6): 445-452.
11. Taveira-DaSilva AM, Jones AM, Julien-Williams P, et al. Long-term effect of sirolimus on serum vascular endothelial growth factor D levels in patients with lymphangioleiomyomatosis. Chest, 2018, 153(1): 124-132.
12. Bissler J J, Kingswood J C, Radzikowska E, et al. Everolimus for angiomyolipoma associated with tuberous sclerosis complex or sporadic lymphangioleiomyomatosis (EXIST-2): a multicentre, randomised, double-blind, placebo-controlled trial. Lancet, 2013, 381(9869): 817-824.
13. Darling TN. Topical sirolimus to treat tuberous sclerosis complex (TSC). JAMA Dermatol, 2018, 154(7): 761-762.
14. Wataya-Kaneda M, Ohno Y, Fujita Y, et al. Sirolimus gel treatment vs placebo for facial angiofibromas in patients with tuberous sclerosis complex: a randomized clinical trial. JAMA Dermatol, 2018, 154(7): 781-788.
15. 中華醫學會呼吸病學分會間質性肺疾病學組, 淋巴管肌瘤病共識專家組, 中國醫學科學院罕見病研究中心, 等. 西羅莫司治療淋巴管肌瘤病專家共識(2018). 中華結核和呼吸雜志, 2019, 42(2): 92-97.
16. Ando K, Kurihara M, Kataoka H, et al. Efficacy and safety of low-dose sirolimus for treatment of lymphangioleiomyomatosis. Respir Investig, 2013, 51(3): 175-183.
17. Bee J, Fuller S, Miller S, et al. Lung function response and side effects to rapamycin for lymphangioleiomyomatosis: a prospective national cohort study. Thorax, 2018, 73(4): 369-375.
18. Yoon HY, Hwang JJ, Kim DS, et al. Efficacy and safety of low-dose Sirolimus in Lymphangioleiomyomatosis. Orphanet J Rare Dis, 2018, 13(1): 204.
19. Xu KF, Tian X, Yang Y, et al. Rapamycin for lymphangioleiomyomatosis: optimal timing and optimal dosage. Thorax, 2018, 73(4): 308-310.
20. Gupta N, Lee H, Young LR, et al. Analysis of the MILES cohort reveals determinants of disease progression and treatment response in lymphangioleiomyomatosis. Eur Respir J, 2019, 53(4): 1802066.
21. Goncharova EA, Goncharov DA, Li H, et al. mTORC2 is required for proliferation and survival of TSC2-null cells. Mol Cell Biol, 2011, 31(12): 2484-2498.
22. Goncharova EA, Goncharov DA, Fehrenbach M, et al. Prevention of alveolar destruction and airspace enlargement in a mouse model of pulmonary lymphangioleiomyomatosis (LAM). Sci Transl Med, 2012, 4(154): 154ra134.
23. Krymskaya VP, Courtwright AM, Fleck V, et al. A phase II clinical trial of the Safety Of Simvastatin (SOS) in patients with pulmonary lymphangioleiomyomatosis and with tuberous sclerosis complex. Respir Med, 2020, 163: 105898.
24. Le K, Steagall WK, Stylianou M, et al. Effect of beta-agonists on LAM progression and treatment. Proc Natl Acad Sci U S A, 2018, 115(5): E944-E953.
25. Lu C, Lee HS, Pappas GP, et al. A phase II clinical trial of an aromatase inhibitor for postmenopausal women with lymphangioleiomyomatosis. Ann Am Thorac Soc, 2017, 14(6): 919-928.
26. Babaei-Jadidi R, Dongre A, Miller S, et al. Mast cell tryptase release contributes to disease progression in lymphangioleiomyomatosis. Am J Respir Crit Care Med, 2021.
27. Liu HJ, Lizotte PH, Du H, et al. TSC2-deficient tumors have evidence of T cell exhaustion and respond to anti-PD-1/anti-CTLA-4 immunotherapy. JCI Insight, 2018, 3(8).
28. Maisel K, Merrilees MJ, Atochina-Vasserman E N, et al. Immune checkpoint ligand PD-L1 is upregulated in pulmonary lymphangioleiomyomatosis. Am J Respir Cell Mol Biol, 2018, 59(6): 723-732.
29. Bendeck MP, Conte M, Zhang M, et al. Doxycycline modulates smooth muscle cell growth, migration, and matrix remodeling after arterial injury. Am J Pathol, 2002, 160(3): 1089-1095.
30. Matsui K, Takeda K, Yu ZX, et al. Role for activation of matrix metalloproteinases in the pathogenesis of pulmonary lymphangioleiomyomatosis. Arch Pathol Lab Med, 2000, 124(2): 267-275.
31. Odajima N, Betsuyaku T, Nasuhara Y, et al. Matrix metalloproteinases in blood from patients with LAM. Respir Med, 2009, 103(1): 124-129.
32. Moses M A, Harper J, Folkman J. Doxycycline treatment for lymphangioleiomyomatosis with urinary monitoring for MMPs. N Engl J Med, 2006, 354(24): 2621-2622.
33. Baldi B G, Ribeiro C C. Doxycycline in lymphangioleiomyomatosis: not all questions are answered. Eur Respir J, 2014, 43(5): 1536-1537.
34. Chang WY, Cane JL, Kumaran M, et al. A 2-year randomised placebo-controlled trial of doxycycline for lymphangioleiomyomatosis. Eur Respir J, 2014, 43(4): 1114-1123.
35. El-Chemaly S, Taveira-Dasilva A, Goldberg HJ, et al. Sirolimus and autophagy inhibition in lymphangioleiomyomatosis: results of a phase I clinical trial. Chest, 2017, 151(6): 1302-1310.
36. El-Chemaly S, Taveira-DaSilva A, Bagwe S, et al. Celecoxib in lymphangioleiomyomatosis: results of a phase I clinical trial. Eur Respir J, 2020, 55(5).
37. Almoosa KF, Ryu JH, Mendez J, et al. Management of pneumothorax in lymphangioleiomyomatosis: effects on recurrence and lung transplantation complications. Chest, 2006, 129(5): 1274-1281.
38. Hayashida M, Seyama K, Inoue Y, et al. The epidemiology of lymphangioleiomyomatosis in Japan: a nationwide cross-sectional study of presenting features and prognostic factors. Respirology, 2007, 12(4): 523-530.
39. 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. Am J Respir Crit Care Med, 2017, 196(10): 1337-1348.
40. Kurihara M, Mizobuchi T, Kataoka H, et al. A total pleural covering for lymphangioleiomyomatosis prevents pneumothorax recurrence. PLoS One, 2016, 11(9): e163637.
41. Suzuki E, Kurihara M, Tsuboshima K, et al. The effects of total pleural covering on pneumothorax recurrence and pulmonary function in lymphangioleiomyomatosis patients without history of pleurodesis or thoracic surgeries for pneumothorax. J Thorac Dis, 2021, 13(1): 113-124.
42. Gonano C, Pasquier J, Daccord C, et al. Air travel and incidence of pneumothorax in lymphangioleiomyomatosis. Orphanet J Rare Dis, 2018, 13(1): 222.
43. Taveira-DaSilva AM, Moss J. Epidemiology, pathogenesis and diagnosis of lymphangioleiomyomatosis. Expert Opin Orphan Drugs, 2016, 4(4): 369-378.
44. Hecimovic A, Jakopovic M, Pavlisa G, et al. Successful treatment of pulmonary and lymphatic manifestations of lymphangioleiomyomatosis with sirolimus. Lymphology, 2015, 48(2): 97-102.
45. Itkin M, McCormack FX. Nonmalignant Adult Thoracic Lymphatic Disorders. Clin Chest Med, 2016, 37(3): 409-420.
46. Taveira-DaSilva AM, Johnson SR, Julien-Williams P, et al. Pregnancy in lymphangioleiomyomatosis: clinical and lung function outcomes in two national cohorts. Thorax, 2020, 75(10): 904-907.
47. Shen L, Xu W, Gao J, et al. Pregnancy after the diagnosis of lymphangioleiomyomatosis (LAM). Orphanet J Rare Dis, 2021, 16(1): 133.
48. Xu KF, Xu W, Liu S, et al. Lymphangioleiomyomatosis. Semin Respir Crit Care Med, 2020, 41(2): 256-268.
49. Ando K, Okada Y, Akiba M, et al. Lung transplantation for lymphangioleiomyomatosis in Japan. PloS one, 2016, 11(1): e146749.
50. Khawar MU, Yazdani D, Zhu Z, et al. Clinical outcomes and survival following lung transplantation in patients with lymphangioleiomyomatosis. J Heart Lung Transplant, 2019, 38(9): 949-955.
51. Salman J, Ius F, Sommer W, et al. Long-term results of bilateral lung transplantation in patients with end-stage pulmonary lymphangioleiomyomatosis. Prog Transplant, 2019, 29(2): 115-121.
52. Groetzner J, Kur F, Spelsberg F, et al. Airway anastomosis complications in de novo lung transplantation with sirolimus-based immunosuppression. J Heart Lung Transplant, 2004, 23(5): 632-638.
53. King-Biggs MB, Dunitz JM, Park SJ, et al. Airway anastomotic dehiscence associated with use of sirolimus immediately after lung transplantation. Transplantation, 2003, 75(9): 1437-1443.
54. El-Chemaly S, Goldberg HJ, Glanville A R. Should mammalian target of rapamycin inhibitors be stopped in women with lymphangioleiomyomatosis awaiting lung transplantation? Expert Rev Respir Med, 2014, 8(6): 657-660.

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淋巴管平滑肌瘤病治療的研究進展

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