Determinants of survival after lung transplantation in telomerase-related gene mutation carriers: A retrospective cohort

被引：14

作者：

Phillips-Houlbracq, Mathilde ^{[1
]}

Mal, Herve ^{[2
,3
,4
]}

Cottin, Vincent ^{[5
]}

Gauvain, Clement ^{[6
]}

Beier, Fabian ^{[7
,8
]}

de Fontbrune, Flore Sicre ^{[9
]}

Sidali, Sabrina ^{[10
]}

Mornex, Jean Francois ^{[5
]}

Hirschi, Sandrine ^{[11
]}

Roux, Antoine ^{[12
]}

Weisenburger, Gaelle ^{[2
,3
,4
]}

Roussel, Arnaud ^{[13
]}

Wemeau-Stervinou, Lidwine ^{[14
]}

Le Pavec, Jerome ^{[15
,16
,17
]}

Pison, Christophe ^{[18
]}

Adam, Sylvain Marchand ^{[19
]}

Froidure, Antoine ^{[20
]}

Lazor, Romain ^{[21
]}

Naccache, Jean-Marc ^{[22
]}

Jouneau, Stephane ^{[23
]}

Nunes, Hilario ^{[24
]}

Reynaud-Gaubert, Martine ^{[25
,26
]}

Le Borgne, Aurelie ^{[27
]}

Boutboul, David ^{[28
]}

Ba, Ibrahima ^{[29
]}

Boileau, Catherine ^{[29
]}

Crestani, Bruno ^{[1
]}

Kannengiesser, Caroline ^{[29
]}

Borie, Raphael ^{[1
]}

机构：

[1] Hop Bichat Claude Bernard, APHP, Serv Pneumol A, Ctr Reference Malad Pulmonaires Rares Site Consti, Paris, France

[2] Univ Paris, Paris, France

[3] INSERM U1152, Paris, France

[4] Hop Bichat Claude Bernard, APHP, Serv Pneumol B, Paris, France

[5] Univ Lyon, Univ Claude Bernard Lyon 1, Ctr Coordonnateur Natl Reference Malad Pulmonaire, Serv Pneumol,Hop Louis Pradel,INRAE,ERN LUNG, Lyon, France

[6] CHU Lille, Serv Doncol, Hop Calmette, Lille, France

[7] Rhein Westfal TH Aachen, Dept Hematol Oncol Hemostaseol & Stem Cell Transp, Aachen, Germany

[8] Ctr Integrated Oncol Aachen Bonn Cologne Dusseldo, Aachen, Germany

[9] Hop St Louis, APHP, Serv Hematol, Paris, France

[10] Hop Beaujon, APHP, Serv Hepatol, Clichy, France

[11] Hop Univ Strasbourg, Ctr Competence Malad Pulmonaires Rares, Serv Pneumol, Strasbourg, France

[12] Hop Foch, Serv Pneumol, Suresnes, France

[13] Hop Bichat Claude Bernard, Serv Chirurg Vasc & Thorac, Paris, France

[14] CHU Lille, Serv Pneumol, Ctr Reference Malad Pulmonaires Rares Site Consti, Lille, France

[15] Grp Hosp St Joseph Marie Lannelongue, Serv Chirurg Thorac Vasc & Transplantat Cardio Pu, Le Plessis Robinson, France

[16] Univ Paris Saclay, Univ Paris Sud, Fac Med, Le Kremlin Bicetre, France

[17] Univ Paris Sud, Hop Marie Lannelongue, INSERM, UMR S 999, Le Plessis Robinson, France

[18] Univ Grenoble Alpes, Serv Hosp Univ Pneumol Physiol, CHU Grenoble Alpes, Pole Thorax & Vaisseaux, Grenoble, France

[19] CHRU Tours, Serv Pneumol, Tours, France

[20] Clin Univ St Luc, Serv Pneumol, Brussels, Belgium

[21] CHU Vaudois, Serv Pneumol, Lausanne, Switzerland

[22] Hop Tenon, Serv Pneumol, Ctr Reference Malad Pulmonaires Rares Site Consti, Paris, France

[23] Univ Rennes 1, Serv Pneumol, Ctr Competences Malad Rares Pulmonaires, Hop Pontchaillou,IRSET UMR 1085, Rennes, France

[24] Hop Avicenne, Ctr Reference Malad Pulmonaires Rares Site Consti, Serv Pneumol, Bobigny, France

[25] CHU Nord, Serv Pneumol, AP HM, Ctr Competences Malad Pulmonaires Rares, Marseille, France

[26] Aix Marseille Univ, MEPHI, IHU Mediterranee Infect, Marseille, France

[27] CHU Toulouse, Serv Pneumol, Ctr Competence Malad Pulmonaires Rares, Hop Larrey, Toulouse, France

[28] Hop St Louis, APHP, Serv Immunopathol Clin, Paris, France

[29] Hop Bichat Claude Bernard, APHP, Lab Genet, Paris, France

来源：

AMERICAN JOURNAL OF TRANSPLANTATION | 2022年 / 22卷 / 04期

关键词：

clinical research; fibrosis; lung disease; lung transplantation; practice; pulmonology; transplant social worker; PULMONARY-FIBROSIS; CLASSIFICATION; DIAGNOSIS; VARIANTS;

D O I：

10.1111/ajt.16893

中图分类号：

R61 [外科手术学];

学科分类号：

摘要：

Carriers of germline telomerase-related gene (TRG) mutations can show poor prognosis, with an increase in common hematological complications after lung transplantation (LT) for pulmonary fibrosis. The aim of this study was to describe the outcomes after LT in recipients carrying a germline TRG mutation and to identify the predictors of survival. In a multicenter cohort of LT patients, we retrospectively reviewed those carrying pathogenic TRG variations (n = 38; TERT, n = 23, TERC, n = 9, RTEL1, n = 6) between 2009 and 2018. The median age at LT was 54 years (interquartile range [IQR] 46-59); 68% were male and 71% had idiopathic pulmonary fibrosis. During the diagnosis of pulmonary fibrosis, 28 (74%) had a hematological disease, including eight with myelodysplasia. After a median follow-up of 26 months (IQR 15-46), 38 patients received LT. The overall post-LT median survival was 3.75 years (IQR 1.8-NA). The risk of death after LT was increased for patients with myelodysplasia (HR 4.1 [95% CI 1.5-11.5]) or short telomere (HR 2.2 [1.0-5.0]) before LT. After LT, all patients had anemia, 66% had thrombocytopenia, and 39% had neutropenia. Chronic lung allograft dysfunction frequency was 29% at 4 years. The present findings support the use of LT in TRG mutation carriers without myelodysplasia. Hematological evaluation should be systematically performed before LT.

引用

页码：1236 / 1244

页数：9

共 21 条

[21] Comparable survival outcome between transplantation from haploidentical donor and matched related donor or unrelated donor for severe aplastic anemia patients aged 40 years and older: A retrospective multicenter cohort study
Zhang, Yuan-Yuan
Mo, Wen-Jian
Zuo, Yang-Yang
Zhou, Ming
Zhang, Xiao-Hui
Wang, Yu
Li, Yu-Miao
Zhang, Yu-Ping
Chen, Yu-Hong
Chen, Xiao-Wei
Mo, Xiao-Dong
Wang, Cai-Xia
Lin, Fan
Huang, Xiao-Jun
Wang, Shun-Qing
Xu, Lan-Ping
CLINICAL TRANSPLANTATION, 2020, 34 (03)

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