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Multiple Solid Organ Transplantation in Telomeropathy

Case Series and Literature Review

Lebeer, Marnix, MD1; Wuyts, Wim A., MD, PhD1; Cassiman, David, MD, PhD2; Laleman, Wim, MD, PhD2; Nevens, Frederik, MD, PhD2; Pirenne, Jacques, MD, PhD3; Monbaliu, Diethard, MD, PhD3; Roskams, Tania, MD, PhD4; Verbeken, Eric K., MD, PhD4; Neyrinck, Arne P., MD, PhD5; Van Raemdonck, Dirk E., MD, PhD6,7; Verleden, Geert M., MD, PhD1,7; Vos, Robin, MD, PhD1,7

doi: 10.1097/TP.0000000000002198
Original Clinical Science—General

Background Solid organ transplantation is a valid treatment option for selected patients with organ failure due to an underlying telomeropathy; however, the feasibility of multiple-organ transplantation if several organs are compromised is unclear.

Methods We describe 2 patients with telomeropathy due to heterozygous telomerase RNA component or telomerase reverse transcriptase mutation, who successfully underwent serial or combined liver and lung transplantation for concurrent liver fibrosis/cirrhosis and pulmonary fibrosis.

Results Despite a challenging posttransplant course, long-term outcomes were favorable, with both patients doing fine now, respectively, 12/20 and 24 months after multiple-organ transplantation.

Conclusions To our knowledge, this is the first report of multiple solid organ transplantation in documented telomeropathy. These cases highlight current difficulties of timely diagnosis, therapeutic approach, and postoperative complications in telomeropathy patients in whom several organs are affected.

Telomerase RNA component (TERC) is the template that telomerase reverse transcriptase (TERT) uses to add telomere-repeats to DNA. The authors describe successful lung-liver transplants in two patients with liver fibrosis/cirrhosis and pulmonary fibrosis from telomeropathies from heterozygous mutations in TERC or TERT.

1 Department of Respiratory Diseases, University Hospitals Leuven; Leuven, Belgium.

2 Department of Gastroenterology and Hepatology, University Hospitals Leuven; Belgium.

3 Department of Abdominal Transplant Surgery and Transplant Coordination, University Hospitals Leuven, Belgium.

4 Department of Histopathology, University Hospitals Leuven; Belgium.

5 Department of Anesthesiology and Algology, University Hospitals Leuven; Belgium.

6 Department of Thoracic Surgery, University Hospitals Leuven and Experimental Thoracic Surgery, KU Leuven; Leuven, Belgium.

7 Division of Respiratory Diseases, Department of Chronic Diseases, Metabolism and Ageing (CHROMETA), KU Leuven; Leuven, Belgium.

Received 28 November 2017. Revision received 15 January 2018.

Accepted 22 January 2018.

R.V. is senior clinical research fellow of the Research Foundation Flanders (FWO), Belgium.

The authors declare no conflicts of interest.

R.V. is supported by a research grant of UZ Leuven, Belgium (STG15/023).

M.L.V. performed data collection, wrote the article, and helped with critical appraisal of the article. W.A.W. is responsible ILD physician of the patients and helped with critical appraisal of the article. D.C. and W.L. are responsible liver physicians, performed endoscopic retrograde cholangiopancreatography and helped with critical appraisal of the article. F.N. is responsible liver physician of the patients and helped with critical appraisal of the article. J.P. and D.M. are responsible liver surgeons and helped with critical appraisal of the article. T.R. and E.K.V. are responsible histopathologists of our liver and lung transplant program and helped with critical appraisal of the article. A.P.N. is responsible anesthesiologist of the patients and helped with the critical appraisal of the article. D.V.R. is the responsible thoracic surgeon and helped with critical appraisal of the article. G.M.V. is the medical director of the transplant program, is the responsible lung transplant physician during pretransplant and posttransplant periods; and helped with critical appraisal of the article. R.V. is the responsible lung transplant physician during pretransplant and posttransplant periods; wrote the article and helped with critical appraisal of the article.

Correspondence: Robin Vos, MD, PhD, Dept. of Respiratory Diseases, Lung Transplantation Unit Herestraat 49, B-3000 Leuven, Belgium. (robin.vos@uzleuven.be).

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