Androgen derivatives improve blood counts and elongate telomere length in adult cryptic dyskeratosis congenita

Medientyp: E-Artikel
Titel: Androgen derivatives improve blood counts and elongate telomere length in adult cryptic dyskeratosis congenita
Beteiligte: Kirschner, Martin; Vieri, Margherita; Kricheldorf, Kim; Ferreira, Monica S. Ventura; Wlodarski, Marcin W.; Schwarz, Michaela; Balabanov, Stefan; Rolles, Benjamin; Isfort, Susanne; Koschmieder, Steffen; Höchsmann, Britta; Panse, Jens; Brümmendorf, Tim H.; Beier, Fabian
Erschienen: Wiley, 2021
Erschienen in: British Journal of Haematology
Sprache: Englisch
DOI: 10.1111/bjh.16997
ISSN: 0007-1048; 1365-2141
Schlagwörter: Hematology
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Beschreibung: <jats:title>Summary</jats:title><jats:p>Dyskeratosis Congenita (DKC) is a systemic disorder caused by mutations resulting in impaired telomere maintenance. Clinical features include bone marrow failure and an increased risk of developing hematological malignancies. There are conflicting data whether androgen derivatives (AD) can elongate telomeres in vivo and whether AD treatment enhances the risk of gaining myelodysplastic syndrome‐related mutations. Seven <jats:italic>TERC</jats:italic> or <jats:italic>TERT</jats:italic>‐mutated DKC patients underwent AD treatment. All patients revealed hematological response. Telomere length of lymphocytes and granulocytes increased significantly and no MDS‐related mutations were detected. Pending longer follow‐up, treatment with AD seems to represent an efficient and safe therapy for DKC patients.</jats:p>

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