Von willebrand factor

VONVENDI ® is indicated in adult and pediatric patients with von Willebrand disease (VWD) for: On-demand treatment and control of bleeding episodes. Perioperative management of bleeding. For adult patients only: Routine prophylaxis to reduce the frequency of bleeding episodes., VONVENDI [von Willebrand factor (recombinant)] is a recombinant von Willebrand factor (rVWF) indicated in adult and pediatric patients with von Willebrand disease (VWD) for: On-demand treatment and control of bleeding episodes. For adult patients only: Routine prophylaxis to reduce the frequency of bleeding episodes.

In VWD patients, VONVENDI acts 1) to promote hemostasis by mediating platelet adhesion to damaged vascular sub-endothelial matrix (e.g., collagen) and platelet aggregation, and 2) as a carrier protein for factor VIII, protecting it from rapid proteolysis. The adhesive activity of VWF depends on the size of its multimers, with larger multimers being the most effective in supporting interactions with collagen and platelet receptors. 1 The binding capacity and affinity of VONVENDI to factor VIII in plasma is comparable to that of endogenous VWF, allowing for VONVENDI to reduce factor VIII clearance. 2

For intravenous use after reconstitution only. On-Demand Treatment and Control of Bleeding Episodes For each bleeding episode, administer the first dose of VONVENDI with an approved recombinant (non-von Willebrand factor containing) factor VIII, if factor VIII baseline levels are below 40% or are unknown. Initial dose is 40 to 80 International Units (IU) per kg body weight (BW). Adjust the dosage based on the extent and location of bleeding. Bleeding Episode Initial Dose Subsequent Dose Minor 40 to 50 IU/kg 40 to 50 IU/kg every 8 to 24 hours Major 50 to 80 IU/kg 40 to 60 IU/kg every 8 to 24 hours for approximately 2 to 3 days Perioperative Management of Bleeding For Elective Surgical Procedure A dose of VONVENDI may be given 12 to 24 hours prior to surgery to allow the endogenous factor VIII levels to increase to at least 30 IU/dL (minor surgery) or 60 IU/dL (major surgery).

VONVENDI is contraindicated in patients who have had life-threatening hypersensitivity reactions to VONVENDI or constituents of the product (tri-sodium citrate-dihydrate, glycine, mannitol, trehalose-dihydrate, polysorbate 80, and hamster or mouse proteins). Do not use in patients who have had life-threatening hypersensitivity reactions to VONVENDI or its components (tri-sodium citrate-dihydrate, glycine, mannitol, trehalose-dihydrate polysorbate 80, and hamster or mouse proteins).

Thromboembolic events have occurred with VONVENDI. Patients with known risk factors for thrombosis are at higher risk. Monitor for early signs of thrombosis and have prophylaxis measures against thromboembolism instituted according to current recommendations. In patients requiring frequent doses of VONVENDI in combination with recombinant factor VIII, monitor plasma levels for FVIII:C because sustained excessive factor VIII plasma levels can increase the risk for thromboembolic events. Hypersensitivity and infusion-related reactions, including anaphylaxis, may occur. Discontinue VONVENDI if hypersensitivity symptoms occur and administer appropriate emergency treatment. Neutralizing antibodies to von Willebrand factor (VWF) and/or factor VIII can occur with VONVENDI.

The most common adverse reactions observed (≥2% of patients) were headache, vomiting, nausea, dizziness and generalized pruritus.

Store at refrigerated temperature 2°C to 8°C (36°F to 46°F) or room temperature not to exceed 30°C (86°F). Do not freeze. Store in the original box and protect from extreme exposure to light.