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Clinical outcomes after lung transplantation for fibrosis in telomerase related genes mutation carriers

Mathilde Phillips Houlbracq Hervé Mal 1 Vincent Cottin 2 Francois Philit 3 Sandrine Hirschi 4 Antoine Roux Lidwine Wemeau-Stervinou 5 Jérôme Le Pavec 6 Christophe Pison 7 Sylvain Marchand-Adam Antoine Froidure 8 Romain Lazor 9 Jean-Marc Naccache 10 Stephane Jouneau 11 Hilario Nunes 12 Martine Reynaud-Gaubert 13, 14 Aurélie Le Borgne Bruno Crestani 15 Caroline Kannengiesser 16 Raphaël Borie 17 
Abstract : Carriers of telomerase related genes (TRG) mutation seem to present a worst prognosis with more common hematological complications after lung transplantation (LT) for pulmonary fibrosis. The aim of this study was to describe the outcomes after LT and identify pre-LT prognosis factors in a multicenter cohort of lung transplant recipients with TRG mutation. We retrospectively reviewed all identified patients with pathogenic TRG mutation (n=38; TERT, n=22, TERC, n=10, RTEL1, n=6) who received LT in France, Switzerland and Belgium between 2009 and 2018. The median age at LT was 54 years (46-59), 70% were male, and 60% had idiopathic pulmonary fibrosis (IPF). At diagnosis of pulmonary fibrosis, 84% had a hematological disease, including 8 with myelodysplasia, and 45% had a liver disease. After a median follow up of 2.2 years (1,2-3,7), 16 received a single LT, 22 a double LT and 2 a combined liver-LT. The overall post-LT median survival was 3.75 years (1.8-NA). Patients with myelodysplasia before LT had an increased risk of death after LT (HR= 4.12 (1.47-11.53) p=0.007). After LT, all patients showed anemia, 70% thrombocytopenia, and 60% neutropenia. Four patients showed severe liver disease: portal hypertension, cirrhosis. Sixteen patients (42%) experienced acute renal failure and 18 (47%) developed chronic renal insufficiency during follow up. Four developed chronic lung allograft dysfunction. Overall survival after LT supports LT in TRG mutation carriers. Careful evaluation at diagnosis, might limit LT indication for patients with established myelodysplasia.
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Submitted on : Friday, April 17, 2020 - 5:14:51 PM
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Mathilde Phillips Houlbracq, Hervé Mal, Vincent Cottin, Francois Philit, Sandrine Hirschi, et al.. Clinical outcomes after lung transplantation for fibrosis in telomerase related genes mutation carriers. European-Respiratory-Society (ERS) International Congress, Sep 2019, madrid, Spain. pp.PA3365, ⟨10.1183/13993003.congress-2019.PA3365⟩. ⟨hal-02546160⟩



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