MULTIPLE SOLID ORGAN TRANSPLANTATION IN TELOMEROPATHY: CASE SERIES AND LITERATURE REVIEW

Background Solid organ transplantation is a valid treatment option for selected patients with organ failure due to an underlying telomeropathy, however, feasibility of multiple-organ transplantation if several organs are compromised is unclear. Methods We describe 2 patients with telomeropathy due to heterozygous TERC or TERT 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. Address for correspondence: Prof. Dr. Robin Vos, Dept. of Respiratory Diseases, Lung Transplantation Unit, Herestraat 49, B-3000 Leuven, Belgium. Tel: + 32 16 341548 Fax: + 32 16 346803. E-mail: robin.vos@uzleuven.be AUTHORSHIP Author's specific contributions: • MLV: performed data collection, wrote the paper; and helped with critical appraisal of the manuscript • WAW: is responsible ILD physician of the patients and helped with critical appraisal of the manuscript • DC and WL: are responsible liver physicians, performed ERCPs and helped with critical appraisal of the manuscript • FN: is responsible liver physician of the patients and helped with critical appraisal of the manuscript • JP and DM: are responsible liver surgeons and helped with critical appraisal of the manuscript • TR and EKV: are responsible histopathologists of our liver and lung transplant program and helped with critical appraisal of the manuscript APN: is responsible anesthesiologist of the patients and helped with critical appraisal of the manuscript • DVR: is responsible thoracic surgeon and helped with critical appraisal of the manuscript • GMV: is medical director of the transplant program, is responsible lung transplant physician during pr- and posttransplant period; and helped with critical appraisal of the manuscript • RV: is responsible lung transplant physician during pre and posttransplant period; wrote the paper and helped with critical appraisal of the manuscript Disclosures: None of the authors of this manuscript have conflicts of interest to disclose regarding the presented data. The authors confirm that that the work described has not been published previously, that it is not under consideration for publication elsewhere, that its publication is approved by all authors and tacitly or explicitly by the responsible authorities where the work was carried out, and that, if accepted, it will not be published elsewhere in the same form in English or in any other language, without the written consent of the copyright holder. Funding: • RV is senior clinical research fellow of the Research Foundation Flanders (FWO), Belgium. • RV is supported by a research grant of UZ Leuven, Belgium (STG15/023) Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.

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