Progress in the application of lung transplantation in pulmonary lymphangioleiomyomatosis
10.12464/j.issn.1674-7445.2024275
- VernacularTitle:肺移植术在肺淋巴管肌瘤病中的应用进展
- Author:
Hanzhou HUANG
1
;
Yongqi CHENG
1
;
Jiaji ZHOU
1
;
Rongguo LU
1
;
Feng LIU
1
;
Mingfeng ZHENG
1
Author Information
1. Wuxi Medical Center, Nanjing Medical University, Wuxi People's Hospital, Department of Thoracic Surgery, the Affiliated Wuxi People's Hospital of Nanjing Medical University, Wuxi 214023, China.
- Publication Type:ReviewArticle
- Keywords:
Pulmonary lymphangioleiomyomatosis;
Lung transplantation;
Mammalian target of rapamycin inhibitor;
Pneumothorax;
Chylothorax;
Dyspnea;
Tuberous sclerosis;
Extracorporeal membrane oxygenation
- From:
Organ Transplantation
2025;16(2):309-314
- CountryChina
- Language:Chinese
-
Abstract:
Pulmonary lymphangioleiomyomatosis is a rare disease characterized by the abnormal proliferation of pulmonary lymphatic smooth muscle cells. It is common in women and often accompanied by recurrent pneumothorax, chylothorax and progressive dyspnea, imaging characterized by diffuse cystic lesions in both lungs. Pulmonary lymphangioleiomyomatosis progresses aggressively and has a very poor prognosis, with a lack of effective medical treatment options in the advanced stages. Lung transplantation is a safe and effective method for the treatment of advanced pulmonary lymphangioleiomyomatosis, which may significantly improve the survival rate and quality of life of patients. The median survival period after surgery can reach 12 years. This article reviews the pathogenesis, diagnosis, treatment of pulmonary lymphangioleiomyomatosis, and the current status and existing problems of lung transplantation in pulmonary lymphangioleiomyomatosis, aiming to provide a reference for the clinical treatment and subsequent research of pulmonary lymphangioleiomyomatosis.
