How is Major Bleeding in Von Willebrand Disease Treated?
How is Major Bleeding in Von Willebrand Disease Treated?
The Big Picture
Von Willebrand Disease (VWD) is a common inherited bleeding disorder. This article reviews treatments available for managing major bleeding episodes in VWD patients.
Contents
- Understanding VWD Treatment Strategies
- The Role of VWF Concentrates
- Additional Therapies to Consider
- Monitoring and Adjusting Treatment
Understanding VWD Treatment Strategies
Managing major bleeding in VWD involves increasing von Willebrand factor (VWF) levels in the blood. This can be achieved through VWF concentrates, which are typically administered in situations where bleeding is severe or life-threatening. DDAVP, another treatment, helps release stored VWF, but is less effective for serious bleeding and is usually reserved for minor cases.
The Role of VWF Concentrates
VWF concentrates are the primary treatment for major bleeding in VWD, helping to stabilize clot formation. These are administered intravenously to quickly raise VWF activity levels, essential for those undergoing major surgery or experiencing life-threatening bleeding. Continuous infusion may be necessary if the patient develops inhibitors, which are antibodies that neutralize the infused VWF.
Additional Therapies to Consider
In addition to VWF concentrates, antifibrinolytic agents like tranexamic acid are used to stabilize clots by preventing their breakdown. These are particularly useful for bleeding in areas with high fibrinolytic activity, such as the uterus. Platelet transfusions might also be necessary for patients with low platelet counts, especially in type 2B VWD.
Monitoring and Adjusting Treatment
Treatment duration is tailored to the individual's needs, often continuing for 5 to 10 days. Monitoring VWF and factor VIII activity is crucial to ensure therapeutic levels are maintained. Adjustments are made based on clinical response and any emerging complications, like thrombosis due to elevated factor VIII levels.
FAQs
What is the main treatment for major bleeding in VWD?
VWF concentrates are the primary treatment for serious bleeding episodes in VWD.
How does DDAVP work in VWD treatment?
DDAVP releases stored VWF but is mostly effective for minor bleeding.
Why are antifibrinolytic agents used in VWD?
They help stabilize clots by preventing their breakdown.
What is the risk with prolonged VWF concentrate use?
Prolonged use may lead to elevated factor VIII levels, increasing thrombosis risk.
Key Takeaways
Choosing the right treatment for major bleeding in VWD is crucial and involves careful monitoring and adjustment.
Additional References
- Nichols WL, Hultin MB, James AH, et al. von Willebrand disease (VWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel report (USA). Haemophilia 2008; 14:171.
- Laffan MA, Lester W, O'Donnell JS, et al. The diagnosis and management of von Willebrand disease: a United Kingdom Haemophilia Centre Doctors Organization guideline approved by the British Committee for Standards in Haematology. Br J Haematol 2014; 167:453.
- Connell NT, Flood VH, Brignardello-Petersen R, et al. ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease. Blood Adv 2021; 5:301.
This article has been reviewed for accuracy by one of the licensed medical doctors working for Doctronic.