Determinants of survival after lung transplantation in telomerase-related gene mutation carriers: A retrospective cohort.
Details
Serval ID
serval:BIB_C879C1EC9A4B
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Determinants of survival after lung transplantation in telomerase-related gene mutation carriers: A retrospective cohort.
Journal
American journal of transplantation
Working group(s)
OrphaLung Network
ISSN
1600-6143 (Electronic)
ISSN-L
1600-6135
Publication state
Published
Issued date
04/2022
Peer-reviewed
Oui
Volume
22
Number
4
Pages
1236-1244
Language
english
Notes
Publication types: Journal Article ; Multicenter Study
Publication Status: ppublish
Publication Status: ppublish
Abstract
Carriers of germline telomerase-related gene (TRG) mutations can show poor prognosis, with an increase in common hematological complications after lung transplantation (LT) for pulmonary fibrosis. The aim of this study was to describe the outcomes after LT in recipients carrying a germline TRG mutation and to identify the predictors of survival. In a multicenter cohort of LT patients, we retrospectively reviewed those carrying pathogenic TRG variations (n = 38; TERT, n = 23, TERC, n = 9, RTEL1, n = 6) between 2009 and 2018. The median age at LT was 54 years (interquartile range [IQR] 46-59); 68% were male and 71% had idiopathic pulmonary fibrosis. During the diagnosis of pulmonary fibrosis, 28 (74%) had a hematological disease, including eight with myelodysplasia. After a median follow-up of 26 months (IQR 15-46), 38 patients received LT. The overall post-LT median survival was 3.75 years (IQR 1.8-NA). The risk of death after LT was increased for patients with myelodysplasia (HR 4.1 [95% CI 1.5-11.5]) or short telomere (HR 2.2 [1.0-5.0]) before LT. After LT, all patients had anemia, 66% had thrombocytopenia, and 39% had neutropenia. Chronic lung allograft dysfunction frequency was 29% at 4 years. The present findings support the use of LT in TRG mutation carriers without myelodysplasia. Hematological evaluation should be systematically performed before LT.
Keywords
Humans, Idiopathic Pulmonary Fibrosis/genetics, Idiopathic Pulmonary Fibrosis/surgery, Lung Transplantation/adverse effects, Male, Middle Aged, Mutation, Retrospective Studies, Telomerase/genetics, clinical research, fibrosis, lung disease, lung transplantation, practice, pulmonology, transplant social worker
Pubmed
Web of science
Create date
11/12/2021 13:59
Last modification date
12/04/2022 6:34