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BLOOD AND BLOOD FORMING ORGANS › ANTIHEMORRHAGICS › VITAMIN K AND OTHER HEMOSTATICS › Blood coagulation factors
von Willebrand factor and coagulation factor VIII in combination
B02BD06
Forms & Strengths
- Lyophilized powder for injection containing human von Willebrand factor (VWF) and coagulation factor VIII (FVIII)
- Potency expressed in international units (IU) of VWF:RCo (ristocetin cofactor) and FVIII activity
- Available in various ratio presentations (e.g., VWF:FVIII 1:1, 2:1, or 2.4:1) with specific IU strengths per vial
Adult Dosing
- Von Willebrand disease (VWD) bleeding/surgery: 40-80 IU VWF:RCo/kg (based on VWF:RCo) initially, followed by 20-40 IU/kg every 8-24 hours as needed
- Hemophilia A prophylaxis/treatment: Dosing based on FVIII units, where 1 IU/kg raises FVIII level by approximately 2 IU/dL
- Surgical coverage in VWD: Pre-operative dose to achieve 100% VWF:RCo and FVIII levels, maintained post-operatively >50% until healing is complete
Pediatric Dosing
- VWD bleeding/surgery: Similar mg/kg or IU/kg dosing as adults, though pediatric clearance may be faster requiring more frequent administration
- Hemophilia A: Dosing calculated based on target FVIII levels and body weight, adjusted for age-related pharmacokinetic differences
Indications
- Treatment and prevention of bleeding in patients with von Willebrand disease (VWD) when desmopressin (DDAVP) is ineffective or contraindicated
- Management of surgical and invasive bleeding episodes in patients with VWD
- Treatment and prophylaxis of bleeding episodes in patients with hemophilia A
Mechanism of Action
- VWF component promotes platelet adhesion to damaged vascular subendothelium and acts as a carrier protein to stabilize coagulation factor VIII
- FVIII component is an essential cofactor for factor IX, accelerating the conversion of factor X toactivated factor X in the intrinsic coagulation pathway
- Restores normal primary hemostasis and secondary coagulation cascade function in deficient states
Contraindications
- Known hypersensitivity to human plasma-derived products, VWF, FVIII, or any excipients
- History of severe anaphylactic or systemic allergic reactions to plasma-derived concentrates
Adverse Reactions
- Development of neutralizing antibodies (inhibitors) against VWF or FVIII
- Hypersensitivity reactions including urticaria, chest tightness, hypotension, and anaphylaxis
- Thromboembolic events, particularly with high doses or in patients with risk factors, due to excessive FVIII accumulation
- Infusion site reactions, headache, dizziness, and flushing
Drug Interactions
- Concomitant use of antifibrinolytics (e.g., tranexamic acid) increases the risk of thrombosis
- No formal drug-drug interaction studies conducted, but mixing with other intravenous medicinal products in the same infusion is generally not recommended
Curated Content: Needs Vetting Before Put to Clinical Use