Determinants of survival after lung transplantation in telomerase-related gene mutation carriers: A retrospective cohort.
clinical research
fibrosis
lung disease
lung transplantation
practice
pulmonology
transplant social worker
Journal
American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons
ISSN: 1600-6143
Titre abrégé: Am J Transplant
Pays: United States
ID NLM: 100968638
Informations de publication
Date de publication:
04 2022
04 2022
Historique:
revised:
01
10
2021
received:
03
03
2021
accepted:
20
10
2021
pubmed:
3
12
2021
medline:
6
4
2022
entrez:
2
12
2021
Statut:
ppublish
Résumé
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.
Identifiants
pubmed: 34854205
doi: 10.1111/ajt.16893
pii: S1600-6135(22)08181-3
doi:
Substances chimiques
Telomerase
EC 2.7.7.49
Types de publication
Journal Article
Multicenter Study
Langues
eng
Sous-ensembles de citation
IM
Pagination
1236-1244Informations de copyright
© 2021 The American Society of Transplantation and the American Society of Transplant Surgeons.
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