von Willebrand disease and angiodysplasia: a wider view of pathogenesis in pursuit of therapy
File(s)
Published version
Author(s)
Crossette-Thambiah, Christina
Randi, Anna M
Laffan, Michael
Type
Journal Article
Abstract
Bleeding in the gastrointestinal tract in patients with von Willebrand disease continues to pose a therapeutic challenge for clinicians. It is associated with significant morbidity and mortality and represents the major unmet need in this disease. Defective angiogenesis in the gut is primarily responsible, resulting in angiodysplastic malformations making bleeding notoriously refractory to standard replacement therapy. A substantial body of evidence now shows that von Willebrand factor has a role in the regulation of angiogenesis but the mechanisms responsible for the formation of vascular malformations remain incompletely understood. Data from the wider field of vascular malformations may lend insight and point to novel therapeutic approaches. Here we review evidence linking von Willebrand factor to angiodysplasia, the associated molecular mechanisms and the implications for therapy.
Date Issued
2025-03-01
Date Acceptance
2024-10-30
Citation
Haematologica: the hematology journal, 2025, 110 (3), pp.588-595
ISSN
0390-6078
Publisher
Ferrata Storti Foundation
Start Page
588
End Page
595
Journal / Book Title
Haematologica: the hematology journal
Volume
110
Issue
3
Copyright Statement
© 2025 Ferrata Storti Foundation Published under a CC BY-NC license
License URL
Identifier
https://www.ncbi.nlm.nih.gov/pubmed/39506901
Subjects
Humans
Angiodysplasia
von Willebrand Diseases
von Willebrand Factor
Animals
Neovascularization, Pathologic
Publication Status
Published
Coverage Spatial
Italy
Date Publish Online
2024-11-07