Desmopressin for bleeding in non-severe hemophilia A: Suboptimal use in a real-world setting.

desmopressin factor VIII hemophilia A hemorrhage treatment

Journal

Research and practice in thrombosis and haemostasis
ISSN: 2475-0379
Titre abrégé: Res Pract Thromb Haemost
Pays: United States
ID NLM: 101703775

Informations de publication

Date de publication:
Aug 2022
Historique:
received: 09 02 2022
revised: 28 05 2022
accepted: 23 06 2022
entrez: 12 9 2022
pubmed: 13 9 2022
medline: 13 9 2022
Statut: epublish

Résumé

Desmopressin is an important treatment option in nonsevere hemophilia A because it has several benefits compared with factor (F) concentrates, including no inhibitor risk and much lower costs. Despite these advantages, data are limited on the real-world use of desmopressin in the treatment of bleeds. To describe the clinical use of desmopressin in relation to other therapeutic modalities in the treatment of bleeding episodes in patients with nonsevere hemophilia A. Patients with nonsevere hemophilia A aged 12-55 years were included from the DYNAMO cohort study. Data on the desmopressin test response and treated bleeding events in the period January 2009 to July 2020 were retrospectively collected from medical files. An adequate desmopressin test response was defined based on a peak FVIII level of ≥30 IU/dl. A total of 248 patients with a median age of 38 years (interquartile range 25-49) were included. An adequate desmopressin test response was documented in 25% and 73% of patients with moderate and mild hemophilia, respectively. In adequate responders, 51% of bleeds were exclusively treated with FVIII concentrates, 24% exclusively with desmopressin, 21% with a combination of both and 4% with other treatments. In 54% of bleeds treated with a single dose of factor concentrates, the expected FVIII level after desmopressin exceeded the level targeted. Most bleeds in patients with an adequate response to desmopressin are treated with factor concentrates. These findings may indicate a suboptimal use of desmopressin and that barriers to the use of desmopressin should be explored.

Sections du résumé

Background UNASSIGNED
Desmopressin is an important treatment option in nonsevere hemophilia A because it has several benefits compared with factor (F) concentrates, including no inhibitor risk and much lower costs. Despite these advantages, data are limited on the real-world use of desmopressin in the treatment of bleeds.
Objective UNASSIGNED
To describe the clinical use of desmopressin in relation to other therapeutic modalities in the treatment of bleeding episodes in patients with nonsevere hemophilia A.
Methods UNASSIGNED
Patients with nonsevere hemophilia A aged 12-55 years were included from the DYNAMO cohort study. Data on the desmopressin test response and treated bleeding events in the period January 2009 to July 2020 were retrospectively collected from medical files. An adequate desmopressin test response was defined based on a peak FVIII level of ≥30 IU/dl.
Results UNASSIGNED
A total of 248 patients with a median age of 38 years (interquartile range 25-49) were included. An adequate desmopressin test response was documented in 25% and 73% of patients with moderate and mild hemophilia, respectively. In adequate responders, 51% of bleeds were exclusively treated with FVIII concentrates, 24% exclusively with desmopressin, 21% with a combination of both and 4% with other treatments. In 54% of bleeds treated with a single dose of factor concentrates, the expected FVIII level after desmopressin exceeded the level targeted.
Conclusion UNASSIGNED
Most bleeds in patients with an adequate response to desmopressin are treated with factor concentrates. These findings may indicate a suboptimal use of desmopressin and that barriers to the use of desmopressin should be explored.

Identifiants

pubmed: 36090159
doi: 10.1002/rth2.12777
pii: S2475-0379(22)02346-9
pmc: PMC9433315
doi:

Types de publication

Journal Article

Langues

eng

Pagination

e12777

Informations de copyright

© 2022 The Authors. Research and Practice in Thrombosis and Haemostasis published by Wiley Periodicals LLC on behalf of International Society on Thrombosis and Haemostasis (ISTH).

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Auteurs

Anne-Fleur Zwagemaker (AF)

Amsterdam UMC University of Amsterdam, Emma Children's Hospital, Pediatric Hematology Amsterdam The Netherlands.

Fabienne R Kloosterman (FR)

Amsterdam UMC University of Amsterdam, Emma Children's Hospital, Pediatric Hematology Amsterdam The Netherlands.

Michiel Coppens (M)

Department of Vascular Medicine Amsterdam University Medical Centers Amsterdam The Netherlands.

Samantha C Gouw (SC)

Amsterdam UMC University of Amsterdam, Emma Children's Hospital, Pediatric Hematology Amsterdam The Netherlands.

Sara Boyce (S)

Department of Haematology University Hospital Southampton Southampton UK.

Catherine N Bagot (CN)

Department of Haematology Glasgow Royal Infirmary Glasgow UK.

Erik A M Beckers (EAM)

Department of Internal Medicine, Division of Hematology, CARIM School for Cardiovascular Diseases Maastricht University Medical Center Maastricht The Netherlands.

Paul Brons (P)

Department of Pediatric Hemato-Oncology Radboud University Medical Center Nijmegen The Netherlands.

Giancarlo Castaman (G)

Department of Oncology, Center for Bleeding Disorders Careggi University Hospital Florence Italy.

Jeroen Eikenboom (J)

Department of Internal Medicine, Division of Thrombosis and Hemostasis Leiden University Medical Center Leiden The Netherlands.

Shannon Jackson (S)

Adult Bleeding Disorders Program of BC - Adult Division St. Paul's Hospital Vancouver British Columbia Canada.

Marieke J H A Kruip (MJHA)

Department of Hematology Erasmus MC, Erasmus University Medical Center Rotterdam The Netherlands.

Frank W G Leebeek (FWG)

Department of Hematology Erasmus MC, Erasmus University Medical Center Rotterdam The Netherlands.

Karina Meijer (K)

Department of Hematology University Medical Center Groningen, University of Groningen Groningen The Netherlands.

Laurens Nieuwenhuizen (L)

Department of Hematology Maxima Medical Center Veldhoven The Netherlands.

Ingrid Pabinger (I)

Clinical Division of Hematology and Hemostaseology Medical University of Vienna Vienna Austria.

Karin Fijnvandraat (K)

Amsterdam UMC University of Amsterdam, Emma Children's Hospital, Pediatric Hematology Amsterdam The Netherlands.
Department of Molecular Cellular Hemostasis Sanquin Research and Landsteiner Laboratory Amsterdam The Netherlands.

Classifications MeSH