Androgen derivatives improve blood counts and elongate telomere length in adult cryptic dyskeratosis congenita
| Autor: | Benjamin Rolles, Tim H. Brümmendorf, Kim Kricheldorf, Martin Kirschner, Michaela Schwarz, Susanne Isfort, Marcin W. Wlodarski, Steffen Koschmieder, Jens Panse, Britta Höchsmann, Margherita Vieri, Fabian Beier, Mónica S. Ventura Ferreira, Stefan Balabanov |
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| Přispěvatelé: | University of Zurich, Kirschner, Martin |
| Rok vydání: | 2020 |
| Předmět: |
Telomer
Adult Male medicine.drug_class 2720 Hematology Blood count 610 Medicine & health Hematological response Telomer Dyskeratosis Congenita Bone marrow failure disorders 03 medical and health sciences 0302 clinical medicine In vivo medicine Humans ddc:610 Telomerase business.industry Bone marrow failure Telomere Homeostasis Hematology Keratose Middle Aged Telomere medicine.disease Androgen Blood Cell Count Increased risk 030220 oncology & carcinogenesis Myelodysplastic Syndromes 10032 Clinic for Oncology and Hematology Mutation Cancer research Androgens RNA Female Dyskeratosis congenita business DDC 610 / Medicine & health 030215 immunology Follow-Up Studies |
| Zdroj: | British journal of haematology : BJHaem 193(3), 669-673 (2021). doi:10.1111/bjh.16997 |
| ISSN: | 1365-2141 |
| DOI: | 10.1111/bjh.16997 |
| Popis: | Summary 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 TERC or TERT‐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. publishedVersion |
| Databáze: | OpenAIRE |
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