Coagulation Factor Viii / Von Willebrand Factor

What it is and how it works

A plasma-derived concentrate containing both coagulation factor VIII and von Willebrand factor (VWF). VWF mediates platelet adhesion to damaged endothelium and acts as a carrier that stabilises factor VIII in the circulation, while factor VIII restores the intrinsic coagulation pathway.

Indications

Prevention and treatment of bleeding, including surgical bleeding, in von Willebrand disease when desmopressin is ineffective or contraindicated; treatment and prophylaxis of bleeding in haemophilia A.

Dosage and administration (summary)

Dosing is individualised by the specialist according to VWF:RCo and factor VIII levels, type of bleeding or surgery and clinical response; given by slow intravenous injection or infusion.

Contraindications

Known hypersensitivity to the active substances or to any excipient.

Warnings and precautions

Hypersensitivity reactions and development of inhibitors to factor VIII or VWF may occur. In von Willebrand disease, sustained high factor VIII levels can increase the risk of thrombosis, so factor VIII activity should be monitored. The possibility of transmission of infectious agents from plasma cannot be totally excluded.

Pregnancy

Use only if clearly indicated; experience in pregnancy is limited.

Storage and supply

Store at 2–8 °C or as specified by the manufacturer (some products allow storage up to 25 °C); do not freeze and protect from light.

Source: reference summary prepared from the EU SmPC/EPAR, BNF and WHO Model Formulary. The approved product information in Iran governs.