Abstract
Lymphangioleiomyomatosis (LAM) is a rare disease that affects women, especially in child-bearing age. Clinical manifestations include angiomyolipoma, pneumothorax, chylothorax, cystic changes of lungs and progressive pulmonary failure. In this article, we report a case of lung transplantation (LuTX) for end stage pulmonary LAM and the treatment of angiomyolipoma showing growth after LuTX resulting in complete remission with combination therapy of everolimus and tacrolimus.
Highlights
Lymphangioleiomyiomatosis (LAM) is a multisystem disease occurring in lung, abdomen and axial lymphatics [1]
Tuberous sclerosis is caused by the mutations of genes TSC1 and TSC2 which regulate the proteins hamartin and tuberin
A comprehensive study examined the effects of the mammalian target of rapamycin (mTOR) inhibitor sirolimus on angiomyolipomas
Summary
Lymphangioleiomyiomatosis (LAM) is a multisystem disease occurring in lung, abdomen and axial lymphatics [1]. This disease is rare—its prevalence is 1 in 1,000,000 in the whole population—and it usually affects young women. Tuberous sclerosis is caused by the mutations of genes TSC1 and TSC2 which regulate the proteins hamartin and tuberin. These proteins regulate their signal by means of mammalian target of rapamycin (mTOR) which con-. (2014) Successful Tacrolimus Therapy Extended by Everolimus in Retroperitoneal Angiomyolipoma after Lung Transplantation: A Case Report. Angiomyolipomas involve the axial lymphatics in the upper retroperitoneum (RP)
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