Skip to Main content Skip to Navigation
Journal articles

Severe hematologic complications after lung transplantation in patients with telomerase complex mutations

Abstract : BACKGROUND: Mutations in the telomerase complex (TERT and TR) are associated with pulmonary fibrosis and frequent hematologic manifestations. The aim of this study was to characterize the prognosis of lung transplantation in patients with TERT or TR mutations. METHODS: Patients with documented TERT or TR mutations who received a lung transplant between 2007 and 2013 in France were identified via an exhaustive search of the lung transplantation network, one expert genetic laboratory, and the clinical research network on rare pulmonary diseases. RESULTS: There were 9 patients (7 men) with TERT (n = 6) or TR (n = 3) mutations who received a single (n = 8) or a double (n = 1) lung transplant for pulmonary fibrosis. Median age was 50 years (range, 35-61 years) at diagnosis and 52 years (range, 37-62 years) at the time of lung transplantation. Thrombocytopenia was present in 7 patients before lung transplantation. After lung transplantation, 6 patients developed myelodysplasia and/or bone marrow failure, directly contributing to death in 4 cases. Anemia was observed in 9 patients, and neutropenia was observed in 3 patients. The median survival after lung transplantation was 214 days (range, 59-1,709 days). CONCLUSIONS: Patients with mutations of the telomerase complex are at high risk of severe hematologic complications after lung transplantation, in particular, bone marrow failure. Specific recommendations should be developed for appropriate guidance regarding hematologic risk assessment before transplantation and management of the post-transplantation immunosuppressive regimen.
Document type :
Journal articles
Complete list of metadatas

Cited literature [36 references]  Display  Hide  Download

https://hal-univ-rennes1.archives-ouvertes.fr/hal-01146848
Contributor : Muriel Cadieu <>
Submitted on : Tuesday, May 19, 2015 - 11:42:57 AM
Last modification on : Wednesday, August 19, 2020 - 11:17:04 AM
Long-term archiving on: : Tuesday, September 15, 2015 - 6:09:27 AM

File

Severe hematological complicat...
Files produced by the author(s)

Identifiers

Citation

Raphael Borie, Caroline Kannengiesser, Sandrine Hirschi, Jérôme Le Pavec, Hervé Mal, et al.. Severe hematologic complications after lung transplantation in patients with telomerase complex mutations. The Journal of Heart and Lung Transplantation, Elsevier, 2015, 34 (4), pp.538--546. ⟨10.1016/j.healun.2014.11.010⟩. ⟨hal-01146848⟩

Share

Metrics

Record views

1049

Files downloads

1602