Clinical outcomes of lung transplant recipients with telomerase mutations.
- Tokman S Division of Pulmonary and Critical Care Medicine, University of California, San Francisco Medical Center, San Francisco, California. Electronic address: sofya.tokman@gmail.com.
- Singer JP Division of Pulmonary and Critical Care Medicine, University of California, San Francisco Medical Center, San Francisco, California.
- Devine MS Division of Pulmonary and Critical Care Medicine, University of Texas Southwestern Medical Center, Dallas, Texas.
- Westall GP Division of Respiratory Medicine, Alfred Hospital, Melbourne, Australia.
- Aubert JD Division of Respiratory Medicine, Lausanne University Hospital, Lausanne, Switzerland.
- Tamm M Division of Respiratory Medicine, Basel University Hospital, Basel, Switzerland.
- Snell GI Division of Respiratory Medicine, Alfred Hospital, Melbourne, Australia.
- Lee JS Division of Pulmonary and Critical Care Medicine, University of California, San Francisco Medical Center, San Francisco, California.
- Goldberg HJ Division of Pulmonary and Critical Care Medicine, Brigham and Women's Hospital, Boston, Massachusetts.
- Kukreja J Division of Cardiothoracic Surgery, University of California, San Francisco Medical Center, San Francisco, California.
- Golden JA Division of Pulmonary and Critical Care Medicine, University of California, San Francisco Medical Center, San Francisco, California.
- Leard LE Division of Pulmonary and Critical Care Medicine, University of California, San Francisco Medical Center, San Francisco, California.
- Garcia CK Division of Pulmonary and Critical Care Medicine, University of Texas Southwestern Medical Center, Dallas, Texas.
- Hays SR Division of Pulmonary and Critical Care Medicine, University of California, San Francisco Medical Center, San Francisco, California.
- 2015-07-15
Published in:
- The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation. - 2015
lung transplantation
pulmonary fibrosis
telomerase mutations
telomere
telomere syndrome
Female
Graft Rejection
Humans
Lung Transplantation
Male
Middle Aged
Mutation
Pulmonary Fibrosis
Retrospective Studies
Telomerase
Treatment Outcome
English
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.
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.
- Language
-
- English
- Open access status
- green
- Identifiers
-
- DOI 10.1016/j.healun.2015.05.002
- PMID 26169663
- Persistent URL
- https://sonar.ch/global/documents/232231
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