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The process of blood coagulation has two distinct phases: primary hemostasis and secondary hemostasis. The hemostatic plug cannot form without both phases occurring. While FVIII has a role in secondary hemostasis, VWF is the key component in primary hemostasis.
Slides 1, 2, and 3 illustrate the crucial role of VWF in primary hemostasis, while Slide 4 shows the process of secondary hemostasis. Slide 5 illustrates how LMW-VWF forms an impaired hemostatic plug.
 
When the vessel is injured, several actions occur to form the initial hemostatic plug:
- VWF unfolds from its inactivated pretzel-like shape, exposing A1 domains (platelet receptors)
- Collagen binds with A3 domains (collagen-binding sites)
HMW-VWF has more A3 domains and A1 domains than LMW-VWF, allowing more bonds to form.1
At the same time, platelets rush to the injury site, forming bonds with A1 domains
On Slide 3, we see how platelet aggregation occurs when platelet thrombi, or bridges, form. The platelet bridges and VWF form layers, known as the initial hemostatic plug, which stops the bleeding at the injury site.1
Secondary hemostasis occurs when a chain reaction of clotting factor proteins occurs (the clotting cascade), leading to additional platelet binding and the release of fibrin. The result is the formation of a stable hemostatic plug.1
In comparison, LMW-VWF, which has few A1 and A3 domains, forms an impaired hemostatic plug, making long-term blood clotting difficult or impossible.

Important Safety Information
Antihemophilic Factor/von Willebrand Factor Complex (Human), Dried, Pasteurized, Humate-P® is indicated in adult patients for treatment and prevention of bleeding in hemophilia A (classical hemophilia). Humate-P® is also indicated in adult and pediatric patients with von Willebrand disease (VWD) for (1) treatment of spontaneous and trauma-induced bleeding episodes, and (2) prevention of excessive bleeding during and after surgery. This applies to patients with severe VWD, and patients with mild and moderate VWD for whom use of desmopressin is known or suspected to be inadequate.
Humate-P® is contraindicated in individuals with a history of anaphylactic or severe systemic response to antihemophilic factor or von Willebrand factor preparations or to any of its components.
Thromboembolic events have been reported in VWD patients receiving coagulation factor replacement, especially in the setting of known risk factors for thrombosis. Caution should be exercised and antithrombotic measures considered.
Humate-P® is derived from human plasma. As with all plasma-derived products, the risk of transmission of infectious agents, including viruses and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent, cannot be completely eliminated.
Although few adverse reactions have been reported the most common are allergic-anaphylactic reactions, including urticaria, chest tightness, rash, pruritus, edema, shock, chills and fever, and hypervolemia. For patients undergoing surgery, the most common adverse reactions are postoperative wound or injection-site bleeding.
Please see accompanying full prescribing information for Humate-P®.
The information provided herein is solely for use by physicians and healthcare professionals in the United States. The CSL Behring product listed may not have been approved in other countries and may not be available everywhere.
Reference
- Data on file, 2005, CSL Behring LLC.
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© CSL Behring 2008. The product information presented on this site is intended for US residents only.
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