Factor XIII combined with recombinant factor VIIa: a new means of treating severe hemophilia A

摘要: Summary. Background: Abnormal thrombin generation is considered the key defect in hemophilia. Conventional treatment seeks to correct this using coagulation factor replacement or bypassing agents, for example recombinant VIIa (rFVIIa). Previous studies demonstrate abnormal FXIII activation patients with essential formation of structurally normal, stable clots. Objectives: The present study challenges hypothesis that hemophilia use plasma-derived (pdFXIII) combination rFVIIa will produce a greater improvement clot stability than promotion alone. Methods: Fourteen individuals severe hemophila A were enrolled. Whole blood was spiked ex vivo buffer, (2 μg mL−1) plus pdFXIII (10 μg mL−1). thromboelastometry assessed stability, after tissue (TF) (0.15 pm) tissue-type plasminogen activator (tPa) (2 nm). primary outcome measure area under elasticity curve (AUEC). Results: and significantly improved as measured by AUEC (P < 0.05) compared Conclusion: resulted superior solely enhancing we suggest increasing alone fails fully dysregulation clot-stabilizing mechanisms associated bleeding disorders. Hemorrhage control may be stabilizing drugs. shows potential novel agent.