Von Willebrand Disease and Angiodysplasia: a wider view of pathogenesis in pursuit of therapy
Bleeding in the GI tract continues to pose a therapeutic challenge for clinicians in patients with Von Willebrand Disease (VWD). It is associated with significant morbidity and mortality and represents the major unmet need in VWD. Defective angiogenesis in the gut is primarily responsible, resultin...
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| Format: | Article |
| Language: | English |
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Ferrata Storti Foundation
2024-11-01
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| Series: | Haematologica |
| Online Access: | https://haematologica.org/article/view/11812 |
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| _version_ | 1850199228674998272 |
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| author | Christina Crossette-Thambiah Anna M. Randi Michael Laffan |
| author_facet | Christina Crossette-Thambiah Anna M. Randi Michael Laffan |
| author_sort | Christina Crossette-Thambiah |
| collection | DOAJ |
| description |
Bleeding in the GI tract continues to pose a therapeutic challenge for clinicians in patients with Von Willebrand Disease (VWD). It is associated with significant morbidity and mortality and represents the major unmet need in VWD. 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 (VWF) 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 VWF to angiodysplasia, the associated molecular mechanisms and the implications for therapy.
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| format | Article |
| id | doaj-art-0d979f3fed14468ba9c31bbdf314bd8f |
| institution | OA Journals |
| issn | 0390-6078 1592-8721 |
| language | English |
| publishDate | 2024-11-01 |
| publisher | Ferrata Storti Foundation |
| record_format | Article |
| series | Haematologica |
| spelling | doaj-art-0d979f3fed14468ba9c31bbdf314bd8f2025-08-20T02:12:41ZengFerrata Storti FoundationHaematologica0390-60781592-87212024-11-01999110.3324/haematol.2024.285244Von Willebrand Disease and Angiodysplasia: a wider view of pathogenesis in pursuit of therapyChristina Crossette-Thambiah0Anna M. Randi1Michael Laffan2Centre for Haematology, Department of Immunology and Inflammation, Imperial College London, UK; Department of Haematology, Imperial College Healthcare NHS Trust, LondonNational Heart and Lung Institute, Imperial CollegeCentre for Haematology, Department of Immunology and Inflammation, Imperial College London, UK; Department of Haematology, Imperial College Healthcare NHS Trust, London Bleeding in the GI tract continues to pose a therapeutic challenge for clinicians in patients with Von Willebrand Disease (VWD). It is associated with significant morbidity and mortality and represents the major unmet need in VWD. 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 (VWF) 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 VWF to angiodysplasia, the associated molecular mechanisms and the implications for therapy. https://haematologica.org/article/view/11812 |
| spellingShingle | Christina Crossette-Thambiah Anna M. Randi Michael Laffan Von Willebrand Disease and Angiodysplasia: a wider view of pathogenesis in pursuit of therapy Haematologica |
| title | Von Willebrand Disease and Angiodysplasia: a wider view of pathogenesis in pursuit of therapy |
| title_full | Von Willebrand Disease and Angiodysplasia: a wider view of pathogenesis in pursuit of therapy |
| title_fullStr | Von Willebrand Disease and Angiodysplasia: a wider view of pathogenesis in pursuit of therapy |
| title_full_unstemmed | Von Willebrand Disease and Angiodysplasia: a wider view of pathogenesis in pursuit of therapy |
| title_short | Von Willebrand Disease and Angiodysplasia: a wider view of pathogenesis in pursuit of therapy |
| title_sort | von willebrand disease and angiodysplasia a wider view of pathogenesis in pursuit of therapy |
| url | https://haematologica.org/article/view/11812 |
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