Clinical outcomes of lung transplant recipients with telomerase mutations.

Détails

ID Serval
serval:BIB_D5C4DB490E81
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Institution
Titre
Clinical outcomes of lung transplant recipients with telomerase mutations.
Périodique
Journal of Heart and Lung Transplantation : the Official Publication of the International Society For Heart Transplantation
Auteur(s)
Tokman S., Singer J.P., Devine M.S., Westall G.P., Aubert J.D., Tamm M., Snell G.I., Lee J.S., Goldberg H.J., Kukreja J., Golden J.A., Leard L.E., Garcia C.K., Hays S.R.
ISSN
1557-3117 (Electronic)
ISSN-L
1053-2498
Statut éditorial
Publié
Date de publication
2015
Peer-reviewed
Oui
Volume
34
Numéro
10
Pages
1318-1324
Langue
anglais
Résumé
BACKGROUND: Successful lung transplantation for patients with pulmonary fibrosis from telomerase mutations may be limited by systemic complications of telomerase dysfunction, including myelosuppression, cirrhosis, and malignancy. We describe clinical outcomes in 14 lung transplant recipients with telomerase mutations.
METHODS: Subjects underwent lung transplantation between February 2005 and April 2014 at 5 transplant centers. Data were abstracted from medical records, focusing on outcomes reflecting post-transplant treatment effects likely to be complicated by telomerase mutations.
RESULTS: The median age of subjects was 60.5 years (interquartile range = 52.0-62.0), 64.3% were male, and the mean post-transplant observation time was 3.2 years (SD ± 2.9). A mutation in telomerase reverse transcriptase was present in 11 subjects, a telomerase RNA component mutation was present in 2 subjects, and an uncharacterized mutation was present in 1 subject. After lung transplantation, 10 subjects were leukopenic and 5 did not tolerate lymphocyte anti-proliferative agents. Six subjects developed recurrent lower respiratory tract infections, 7 developed acute cellular rejection (A1), and 4 developed chronic lung allograft dysfunction. Eight subjects developed at least 1 episode of acute renal failure and 10 developed chronic renal insufficiency. In addition, 3 subjects developed cancer. No subjects had cirrhosis. At data censorship, 13 subjects were alive.
CONCLUSIONS: The clinical course for lung transplant recipients with telomerase mutations is complicated by renal disease, leukopenia with intolerance of lymphocyte anti-proliferative agents, and recurrent lower respiratory tract infections. In contrast, cirrhosis was absent, acute cellular rejection was mild, and development of chronic lung allograft dysfunction was comparable to other lung transplant recipients. Although it poses challenges, lung transplantation may be feasible for patients with pulmonary fibrosis from telomerase mutations.
Mots-clé
Female, Graft Rejection, Humans, Lung Transplantation, Male, Middle Aged, Mutation, Pulmonary Fibrosis/diagnosis, Pulmonary Fibrosis/genetics, Retrospective Studies, Telomerase/genetics, Treatment Outcome
Pubmed
Web of science
Création de la notice
17/11/2015 18:34
Dernière modification de la notice
20/08/2019 16:55
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