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Effects of nandrolone decanoate on telomere length and clinical outcome in patients with telomeropathies: a prospective trial.
Clé, Diego V; Catto, Luiz Fernando B; Gutierrez-Rodrigues, Fernanda; Donaires, Flávia S; Pinto, Andre L; Santana, Barbara A; Darrigo, Luiz Guilherme; Valera, Elvis T; Koenigkam-Santos, Marcel; Baddini-Martinez, José; Young, Neal S; Martinez, Edson Z; Calado, Rodrigo T.
Afiliação
  • Clé DV; Department of Medical Imaging, Hematology, and Oncology, Ribeirão Preto Medical School, University of São Paulo, Brazil. dvcle@hcrp.usp.br.
  • Catto LFB; Department of Medical Imaging, Hematology, and Oncology, Ribeirão Preto Medical School, University of São Paulo, Brazil.
  • Gutierrez-Rodrigues F; Hematology Branch, National Heart, Lung, and Blood Institute, National Institute of Health, Bethesda, Maryland, USA.
  • Donaires FS; Department of Medical Imaging, Hematology, and Oncology, Ribeirão Preto Medical School, University of São Paulo, Brazil.
  • Pinto AL; Department of Medical Imaging, Hematology, and Oncology, Ribeirão Preto Medical School, University of São Paulo, Brazil.
  • Santana BA; Department of Medical Imaging, Hematology, and Oncology, Ribeirão Preto Medical School, University of São Paulo, Brazil.
  • Darrigo LG; Department of Pediatrics, Ribeirão Preto Medical School, University of São Paulo, Brazil.
  • Valera ET; Department of Pediatrics, Ribeirão Preto Medical School, University of São Paulo, Brazil.
  • Koenigkam-Santos M; Department of Medical Imaging, Hematology, and Oncology, Ribeirão Preto Medical School, University of São Paulo, Brazil.
  • Baddini-Martinez J; Department of Internal Medicine, Ribeirão Preto Medical School, University of São Paulo, Brazil.
  • Young NS; Hematology Branch, National Heart, Lung, and Blood Institute, National Institute of Health, Bethesda, Maryland, USA.
  • Martinez EZ; Department of Social Medicine, Ribeirão Preto Medical School, University of São Paulo, Brazil.
  • Calado RT; Department of Medical Imaging, Hematology, and Oncology, Ribeirão Preto Medical School, University of São Paulo, Brazil.
Haematologica ; 108(5): 1300-1312, 2023 05 01.
Article em En | MEDLINE | ID: mdl-36579443
Androgens have been reported to elongate telomeres in retrospective and prospective trials with patients with telomeropathies, mainly with bone marrow failure. In our single-arm prospective clinical trial (clinicaltrials gov. Identifier: NCT02055456), 17 patients with short telomeres and/or germline pathogenic variants in telomere biology genes associated with at least one cytopenia and/or radiologic diagnosis of interstitial lung disease were treated with 5 mg/kg of intramuscular nandrolone decanoate every 15 days for 2 years. Ten of 13 evaluable patients (77%) showed telomere elongation at 12 months by flow-fluorescence in situ hybridization (average increase, 0.87 kb; 95% confidence interval: 0.20-1.55 kb; P=0.01). At 24 months, all ten evaluable patients showed telomere elongation (average increase, 0.49 kb; 95% confidence interval: 0.24-1.23 kb; P=0.18). Hematologic response was achieved in eight of 16 patients (50%) with marrow failure at 12 months, and in ten of 16 patients (63%) at 24 months. Seven patients had interstitial lung disease at baseline, and two and three had pulmonary response at 12 and 24 months, respectively. Two patients died due to pulmonary failure during treatment. In the remaining evaluable patients, the pulmonary function remained stable or improved, but showed consistent decline after cessation of treatment. Somatic mutations in myeloid neoplasm-related genes were present in a minority of patients and were mostly stable during drug treatment. The most common adverse events were elevations in liver function test levels in 88%, acne in 59%, and virilization in 59%. No adverse events grade ≥4 was observed. Our findings indicate that nandrolone decanoate elongates telomeres in patients with telomeropathies, which correlated with clinical improvement in some cases and tolerable adverse events.
Assuntos

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Doenças Pulmonares Intersticiais Tipo de estudo: Prognostic_studies / Risk_factors_studies Limite: Humans Idioma: En Revista: Haematologica Ano de publicação: 2023 Tipo de documento: Article País de afiliação: Brasil País de publicação: Itália

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Doenças Pulmonares Intersticiais Tipo de estudo: Prognostic_studies / Risk_factors_studies Limite: Humans Idioma: En Revista: Haematologica Ano de publicação: 2023 Tipo de documento: Article País de afiliação: Brasil País de publicação: Itália