Lung transplantation for lymphangioleiomyomatosis: a Brazilian centre experience
Lymphangioleiomyomatosis (LAM) is a rare low-grade neoplasm with lung cysts formation. Some patients with progressive disease need lung transplantation (LT). Herein we describe clinical characteristics and post operative outcomes of LT in LAM patients at a Brazilian reference centre. Our retrospective study included 11 LAM patients submitted to LT at University of Sao Paulo from 2003 to 2016 (91% double-lung). Mean follow-up after LT was 44 months. Mean age at LT was 43 ± 7 years. Preoperatively, patients presented dyspnea (100%), supplemental oxygen use (91%), pneumothorax (36%) and pulmonary hypertension (36%). One patient underwent pleurodesis and none presented chylothorax. Sirolimus was used by 36% of patients before LT. FEV1 was 28 ± 14% and DLCO 30 ± 18% of predicted. There were no intraoperative complications, whereas one patient required cardiopulmonary bypass due persistent hypoxemia. Post operatively, 18% of patients had chylothorax; 82% presented at least one episode of acute rejection, and chronic lung allograft syndrome occurred in 45%. At least one pulmonary infection occurred in 55% of patients, mainly due to Aspergillus sp. After LT, we found no LAM recurrence, episode of malignant neoplasm or retransplantation. There were 3 deaths (27%) and the survival rate at 5 years after LT was 77% (fig 1). Based on our data, LT is a feasible therapeutic option for patients with end-stage LAM, with favorable prognosis.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Oliveira, M., Guedes Baldi, B., Naoyuki Samano, M., Vidal Campos, S., Henrique Oliveira Braga Teixeira, R., Pasqualini Minguini, I., Burlina, R., Zinoni Silva Pato, E., Roberto Ribeiro Carvalho, C., Nathan Costa, A. Tags: Diffuse Parenchymal Lung Disease Source Type: research
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