Jivi

The factor VIII/von Willebrand factor complex consists of two molecules (factor VIII and von Willebrand factor) with different physiological functions. When infused into a patient with haemophilia, factor VIII binds to patient’s von Willebrand factor. Activated factor VIII acts as a cofactor for activated factor IX, accelerating the conversion of factor X to activated factor X.
Activated factor X converts prothrombin into thrombin. Thrombin then converts fibrinogen into fibrin and a clot can be formed. Haemophilia A is a X-chromosomal hereditary disorder of blood coagulation due to decreased levels or absence of factor VIII:C that results in bleeding into joints, muscles or internal organs, either spontaneously or as a result of accidental or surgical trauma. By replacement therapy the plasma levels of factor VIII are increased, thereby enabling a temporary correction of the factor deficiency and correction of the bleeding tendencies.
Damoctocog alfa pegol is a PEGylated B-domain deleted recombinant human coagulation factor VIII (PEG-BDD-rFVIII). Site-specific PEGylation reduces clearance of factor VIII resulting in an extended half-life while maintaining the normal functions of the B-domain deleted rFVIII molecule. Damoctocog alfa pegol does not contain von Willebrand factor.