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J Heart Lung Transplant. 2015 Oct;34(10):1318-24. doi: 10.1016/j.healun.2015.05.002. Epub 2015 May 11.

Clinical outcomes of lung transplant recipients with telomerase mutations.

Author information

1
Division of Pulmonary and Critical Care Medicine, University of California, San Francisco Medical Center, San Francisco, California. Electronic address: sofya.tokman@gmail.com.
2
Division of Pulmonary and Critical Care Medicine, University of California, San Francisco Medical Center, San Francisco, California.
3
Division of Pulmonary and Critical Care Medicine, University of Texas Southwestern Medical Center, Dallas, Texas.
4
Division of Respiratory Medicine, Alfred Hospital, Melbourne, Australia.
5
Division of Respiratory Medicine, Lausanne University Hospital, Lausanne, Switzerland.
6
Division of Respiratory Medicine, Basel University Hospital, Basel, Switzerland.
7
Division of Pulmonary and Critical Care Medicine, Brigham and Women's Hospital, Boston, Massachusetts.
8
Division of Cardiothoracic Surgery, University of California, San Francisco Medical Center, San Francisco, California.

Abstract

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.

KEYWORDS:

lung transplantation; pulmonary fibrosis; telomerase mutations; telomere; telomere syndrome

PMID:
26169663
PMCID:
PMC5382798
DOI:
10.1016/j.healun.2015.05.002
[Indexed for MEDLINE]
Free PMC Article

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