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Lung transplantation in telomerase mutation carriers with pulmonary fibrosis

Leann L. Silhan, Pali D. Shah, Daniel C. Chambers, Laurie D. Snyder, Gerdt C. Riise, Christa L. Wagner, Eva Hellström-Lindberg, Jonathan B. Orens, Juliette F. Mewton, Sonye K. Danoff, Murat O. Arcasoy, Mary Armanios
European Respiratory Journal 2014; DOI: 10.1183/09031936.00060014
Leann L. Silhan
1Dept of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA
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Pali D. Shah
1Dept of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA
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Daniel C. Chambers
2Dept of Medicine, The Prince Charles Hospital, Brisbane, Australia
3The University of Queensland, Queensland Lung Transplant Service, The Prince Charles Hospital Brisbane, Australia
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Laurie D. Snyder
4Dept of Medicine, Duke University School of Medicine, Durham, NC, USA
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Gerdt C. Riise
5Sahlgrenska University Hospital, Gothenburg, Sweden
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Christa L. Wagner
6Dept of Oncology, Johns Hopkins University School of Medicine, Baltimore, MD, USA
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Eva Hellström-Lindberg
7Dept of Medicine, Karolinska Institute, Huddinge, Sweden
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Jonathan B. Orens
1Dept of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA
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Juliette F. Mewton
8The Prince Charles Hospital Renal Services, Brisbane, Australia
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Sonye K. Danoff
1Dept of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA
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Murat O. Arcasoy
4Dept of Medicine, Duke University School of Medicine, Durham, NC, USA
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Mary Armanios
6Dept of Oncology, Johns Hopkins University School of Medicine, Baltimore, MD, USA
9Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins University School of Medicine, Baltimore, MD, USA
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Abstract

Lung transplantation is the only intervention that prolongs survival in idiopathic pulmonary fibrosis (IPF). Telomerase mutations are the most common identifiable genetic cause of IPF, and at times, the telomere defect manifests in extrapulmonary disease such as bone marrow failure. The relevance of this genetic diagnosis for lung transplant management has not been examined.

We gathered an international series of telomerase mutation carriers who underwent lung transplant in the USA, Australia and Sweden.

The median age at transplant was 52 years. Seven recipients are alive with a median follow-up of 1.9 years (range 6 months to 9 years); one died at 10 months. The most common complications were haematological, with recipients requiring platelet transfusion support (88%) and adjustment of immunosuppressives (100%). Four recipients (50%) required dialysis for tubular injury and calcineurin inhibitor toxicity. These complications occurred at significantly higher rates relative to historic series (p<0.0001).

Our observations support the feasibility of lung transplantation in telomerase mutation carriers; however, severe post-transplant complications reflecting the syndromic nature of their disease appear to occur at higher rates. While these findings need to be expanded to other cohorts, caution should be exercised when approaching the transplant evaluation and management of this subset of pulmonary fibrosis patients.

Abstract

Telomerase mutation carriers with IPF may be prone to complications from their underlying telomere syndrome after LTx http://ow.ly/wmy6P

  • Received February 5, 2014.
  • Accepted April 25, 2014.
  • © ERS

ERJ Open articles are open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.

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European Respiratory Journal: 61 (1)
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Lung transplantation in telomerase mutation carriers with pulmonary fibrosis
Leann L. Silhan, Pali D. Shah, Daniel C. Chambers, Laurie D. Snyder, Gerdt C. Riise, Christa L. Wagner, Eva Hellström-Lindberg, Jonathan B. Orens, Juliette F. Mewton, Sonye K. Danoff, Murat O. Arcasoy, Mary Armanios
European Respiratory Journal Jan 2014, erj00600-2014; DOI: 10.1183/09031936.00060014

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Lung transplantation in telomerase mutation carriers with pulmonary fibrosis
Leann L. Silhan, Pali D. Shah, Daniel C. Chambers, Laurie D. Snyder, Gerdt C. Riise, Christa L. Wagner, Eva Hellström-Lindberg, Jonathan B. Orens, Juliette F. Mewton, Sonye K. Danoff, Murat O. Arcasoy, Mary Armanios
European Respiratory Journal Jan 2014, erj00600-2014; DOI: 10.1183/09031936.00060014
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