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


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 (2020). Androgen derivatives improve blood counts and elongate telomere length in adult cryptic dyskeratosis congenita. British Journal of Haematology:Epub ahead of print.

Abstract

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.

Abstract

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.

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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Clinic for Oncology and Hematology
Dewey Decimal Classification:610 Medicine & health
Scopus Subject Areas:Health Sciences > Hematology
Language:English
Date:3 August 2020
Deposited On:20 Oct 2020 09:58
Last Modified:21 Oct 2020 20:00
Publisher:Wiley-Blackwell Publishing, Inc.
ISSN:0007-1048
OA Status:Hybrid
Free access at:Publisher DOI. An embargo period may apply.
Publisher DOI:https://doi.org/10.1111/bjh.16997
PubMed ID:32744739

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