Clinical, economic, and health-related quality of life burden associated with von Willebrand disease in adults and children: Systematic and targeted literature reviews.


Journal

Haemophilia : the official journal of the World Federation of Hemophilia
ISSN: 1365-2516
Titre abrégé: Haemophilia
Pays: England
ID NLM: 9442916

Informations de publication

Date de publication:
Mar 2023
Historique:
revised: 07 04 2022
received: 17 11 2021
accepted: 26 08 2022
pubmed: 13 9 2022
medline: 21 3 2023
entrez: 12 9 2022
Statut: ppublish

Résumé

Debilitating clinical complications in von Willebrand disease (VWD) can affect health-related quality of life (HRQoL), increase healthcare costs and cause long-lasting consequences. However, the magnitude of these burdens needs to be more fully explored. To estimate the prevalence and burden of clinical complications, the impact on HRQoL and the economic burden associated with VWD. Embase Among 16 studies assessing clinical complications in VWD, the most prevalent bleeding symptoms were menorrhagia (2%-95% [n = 7 studies]), epistaxis (12%-80% [n = 6]) and easy bruising (46%-65% [n = 2]). Among 17 studies evaluating HRQoL, the most common assessment scales were the generic SF-36 (n = 8 studies) and the EQ-5D (n = 2). Bleeding symptoms were associated with reduced QoL in six of seven studies, and of six studies evaluating treatment impact, four reported improvements in one or more HRQoL components. Among 25 studies on cost and resource use, key observations included higher post-surgery healthcare costs in VWD versus non-VWD patients (n = 1 study) and higher costs and resource use in VWD patients with bleeding complications versus those without (n = 1). Although limited, available evidence suggests that VWD patients experience a high burden of clinical complications, reduced QoL and high healthcare costs. Haemarthrosis is more common in severe VWD than is often assumed, and bleeds (including haemarthrosis) can reduce QoL. Research efforts to improve QoL and other outcomes should be prioritized.

Identifiants

pubmed: 36097135
doi: 10.1111/hae.14655
doi:

Substances chimiques

von Willebrand Factor 0

Types de publication

Journal Article Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

411-422

Subventions

Organisme : CSL Behring

Informations de copyright

© 2022 John Wiley & Sons Ltd.

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Auteurs

Giancarlo Castaman (G)

Centre for Bleeding Disorders and Coagulation, Department of Oncology, Careggi University Hospital, Firenze, Italy.

Olga Katsarou (O)

Blood Transfusion Centre, National Reference Centre for Congenital Bleeding Disorders, Laiko General Hospital, Athens, Greece.

Nathalie Jansen (N)

CSL Behring, Hattersheim am Main, Germany.

Sandra Santos (S)

CSL Behring, Hattersheim am Main, Germany.

Ginés Escolar (G)

Department of Haematopathology, Hospital Clinic of Barcelona, Barcelona, Spain.

Erik Berntorp (E)

Clinical Coagulation Research Unit, Lund University, Malmö, Sweden.

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