Blutungsmanagement: rFVIIIFc bei Hämophilie A und Lebertransplantation

Abstract

Abstract Background In patients with severe hemophilia A prolonged bleeding may occur even in cases of minor trauma or surgery. Objective To investigate the feasibility and efficacy of a recombinant extended half-life (EHL) FVIII concentrate for perioperative bleeding management in a patient with severe hemophilia A undergoing liver transplantation. Material and methods Prior to transplantation FVIII activity and perioperatively required FVIII supply were estimated. In an individualized approach efmoroctocog alfa was supplemented if the intrinsic clotting time in the thrombelastometry was > 170 s. Results The patient perioperatively received a total of 28,000 IU efmoroctocog alfa. No signs of hemorrhage or complications were detected and no further intervention was necessary. Conclusion The present case demonstrates that the use of an EHL FVIII is suitable for a successful perioperative bleeding control even in hemophilia patients at a high bleeding risk during major surgery. Due to the EHL constant FVIII levels could be achieved with relatively few injections. In order to confirm the obtained results, more real-world data in different operative settings are essential. Further research is needed on the use of thrombelastometry to guide substitution of factor VIII perioperatively.