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What is Acquired von Willebrand Syndrome?
Published: Apr 03, 2024
Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder that isn't inherited, unlike its more common cousin, von Willebrand disease. Understanding the causes and implications of AVWS can help manage its complex symptoms.
Understanding AVWS
AVWS occurs when there is a deficiency or dysfunction of the von Willebrand factor (VWF), a crucial protein for blood clotting. Unlike inherited von Willebrand disease, AVWS is linked to other underlying health conditions. It can be triggered by diseases that require antithrombotic therapy, such as heart disease, or by certain cancers and autoimmune disorders.
Mechanisms of AVWS
The reduction in VWF in AVWS can happen through various mechanisms, both immune and non-immune. Immune-related reductions often involve autoantibodies that attack the VWF. Non-immune mechanisms might include increased shear stress, which causes the VWF to break down more rapidly, or adsorption, where VWF binds to cells or proteins and gets removed from circulation.
Consequences and Risks
The main risk of AVWS is an increased chance of bleeding, especially if VWF levels fall below certain thresholds. Bleeding can occur in mucosal areas or internally, and some patients also experience reduced levels of another clotting factor, factor VIII. The presence of certain antibodies can increase the severity of bleeding.
Frequently Asked Questions
AVWS can be triggered by underlying conditions like cancer, heart disease, or autoimmune disorders.
No, AVWS is acquired and not inherited.
AVWS increases the risk of bleeding by reducing von Willebrand factor levels.
Key Takeaways
Understanding AVWS and its underlying causes is crucial for effective management and treatment.
References
- Wagner DD. Cell biology of von Willebrand factor. Annu Rev Cell Biol 1990; 6:217.
- Veyradier A, Jenkins CS, Fressinaud E, Meyer D. Acquired von Willebrand syndrome: from pathophysiology to management. Thromb Haemost 2000; 84:175.
This article has been reviewed for accuracy by one of the licensed medical doctors working for Doctronic. Always discuss health information with your healthcare provider.
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