Recurrence of lymphangioleiomyomatosis: Nine years after a bilateral lung transplantation  

作　　者：Khawaja S Zaki Zahra Aryan Atul C Mehta Olufemi Akindipe Marie Budev 

机构地区：[1]Respiratory Institute, Cleveland Clinic [2]Lerner College of Medicine, Bouncore Family Endowed Chair in Lung Transplantation, Cleveland Clinic

出　　处：《World Journal of Transplantation》2016年第1期249-254,共6页世界移植杂志

摘　　要：Lymphangioleiomyomatosis(LAM) is a rare, slowly progressive lethal lung disease primary afflicting young women. LAM is characterized by proliferation of abnormal smooth muscle cells that target the lungs, causing cystic destruction and eventual respiratory failure leading to death. Recent ten year mortality due to end stage LAM has been reported to be approximately 10%-20%, but may vary. The decline in lung function in LAM is gradual, occurring at a rate of about 3% to 15% per year but can vary from patient to patient. But recently therapy with mammalian target of rapamycin(m TOR) inhibitors such as sirolimus has shown promising results in the stabilization of lung function and reduction of chylous effusions in LAM. Lung transplantation is a viable option for patients who continue to have decline in lung function despite m TOR therapy. Unique issues that may occur post-transplant in a recipient with LAM include development of chylous effusion and a risk of recurrence. We describe a case of LAM recurrence in a bilateral lung transplant recipient who developed histological findings of LAM nine years after transplantation.Lymphangioleiomyomatosis(LAM) is a rare, slowly progressive lethal lung disease primary afflicting young women. LAM is characterized by proliferation of abnormal smooth muscle cells that target the lungs, causing cystic destruction and eventual respiratory failure leading to death. Recent ten year mortality due to end stage LAM has been reported to be approximately 10%-20%, but may vary. The decline in lung function in LAM is gradual, occurring at a rate of about 3% to 15% per year but can vary from patient to patient. But recently therapy with mammalian target of rapamycin(m TOR) inhibitors such as sirolimus has shown promising results in the stabilization of lung function and reduction of chylous effusions in LAM. Lung transplantation is a viable option for patients who continue to have decline in lung function despite m TOR therapy. Unique issues that may occur post-transplant in a recipient with LAM include development of chylous effusion and a risk of recurrence. We describe a case of LAM recurrence in a bilateral lung transplant recipient who developed histological findings of LAM nine years after transplantation.

关 键 词：LYMPHANGIOLEIOMYOMATOSIS MAMMALIAN target of RAPAMYCIN inhibitors LUNG transplantation SIROLIMUS LUNG rejection 

分 类 号：R[医药卫生]