Mayo clinic experience of lung transplantation in pulmonary lymphangioleiomyomatosis

Respiratory Medicine
Kamonpun UssavarungsiCharles D Burger

Abstract

Lymphangioleiomyomatosis (LAM) is a rare, cystic lung disease that generally results in progressive decline in lung function. Despite advancement of pharmacological therapy for LAM, lung transplantation remains an important option for women with end-stage LAM. Patients with LAM undergoing lung transplantation at the Mayo Clinic campuses in Rochester, Minnesota and Jacksonville, Florida since 1995 were retrospectively reviewed. Overall, 12 women underwent lung transplantation. Nine of 12 (75%) underwent double lung transplant. The mean age was 42 ± 8 years at the time of transplant. One patient (8%) had a chylothorax and 7 (58%) had recurrent pneumothoraces, 4 (33%) of which required pleurodesis. All had diffuse, cystic lung disease on chest CT consistent with LAM which was confirmed in the explant of all patients. The average length of ICU and hospital stays were 5 ± 4 and 19 ± 19 days, respectively. Mild to moderate anastomotic ischemia was evident in all patients but resolved with time. No patient was treated with sirolimus pre-transplant. Seven patients received sirolimus post-transplant; however, clinical benefit was documented in only 2 patients, 1 of which was treated for large retroperitoneal cysts with ureteral obstruct...Continue Reading

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Citations

Jun 24, 2017·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·Stephen J MurphyEva M Carmona
Jun 28, 2018·F1000Research·Kai-Feng XuJay H Ryu
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Aug 31, 2021·The Lancet. Respiratory Medicine·Cormac McCarthyFrancis X McCormack

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