Freeze-dried activated human blood coagulation factor VII concentrate containing factor X(KM)

Haemophilia A (HA) is a common inherited haemorrhagic disorder that results from a deficiency or defect in the protein factor (F)VIII procoagulantAccording to the recommended guidelines of the on-demand BPA therapy with emicizumab, aPCC (1.3 IU/mL in plasma or .65 IU/mL in whole blood, corresponding to 50 IU/kg) and rFVIIa(2.2 μg/mL in plasma or 1.1 μg/mL in whole blood, corresponding to 90 μg/kg) were added to the blood samples.To assess the coagulation potential of pd-FVIIa/FX together with emicizumab, pd-FVIIa/FX (.75, 1.5 or 3.0 μg/mL; 30, 60 or 120 μg/kg as FVIIa), rFVIIa (2.2 μg/mL; 90 μg/kg) or aPCC (1.3 IU/mL; 50 IU/kg), was added to FVIII-depleted plasma spiked with emicizumab (50 μg/mL).The peak thrombin level in the presence of emicizumab with pd-FVIIa/FX at various concentrations increased dose-dependently, and their coagulation potentials maintained belowor within the normal range.The effects of BPA spiking on global haemostasis in whole blood and plasma samples from three PwHA-Inh receiving emicizumab prophylaxis.These results demonstrated that the coagulant potentials improved sufficiently in the co-presence of pd-FVIIa/FX in emicizumab-treated PwHA-Inh.The present study was performed using a small sample size.Nevertheless, we demonstrated that the concomitant use of emicizumab and pd-FVIIa/FX could be useful for managing breakthrough bleeding and surgeries in PwHA-Inh and PwAHA.