Contributions
Abstract: PB1743
Type: Publication Only
Background
Based on reports addressing hemophilia B patients bleed less common and less intensively than hemophilia A, it has been expected that the hemostatic level of factor IX (FIX) activity can be lowered than that of factor VIII (FVIII) activity.
Aims
We compared the hemostatic efficacy of the different hemostatic level of FIX and FVIII activity using global hemostatic assay.
Methods
A total of 17 severe hemophilia patients without inhibitor, aged more than 15 years old were subjected; 12 hemophilia A patients and 7 hemophilia B patients. Factor concentrates were injected to reach the target activity of 60% in hemophilia A and 40% in hemophilia B which is given by Korean health insurance guideline. All patients were in non-bleeding state and kept the wash-out period of 3 days for hemophilia A and 5 days of hemophilia B. Before and on 15 minutes after injections, we conducted one-stage factor assay, thrombin generation assay (TGA), thromboelastography (TEG) and clot-wave form analysis (CWA).
Results
Median ages of hemophilia A and hemophilia B patients were 28 and 33 years old. Baseline FVIII:C and FIX:C were 0.6% and 1.8% and they rose after injection rose to 70.8% and 49.8%. The dosage of FVIII concentrates and recombinant FIX concentrates were 28.4 IU/kg and 50.7 IU/kg. In-vivo recovery (IVR) in hemophilia A and hemophilia B patients recorded 2.43 %/U/kg and 0.91 %/U/kg. Peak thrombin of FVIII and FIX were 451.3 nM and 376.6 nM (P=0.108, normal range, 458 nM±60). TEG index of FVIII and FIX were -1.60 and -3.77 (P=0.004, normal range, -2~+2). MIN2 of CWA of FVIII and FIX were 0.62 and 0.59 (P=1.000).
Conclusion
Global hemostatic assay indicates even though IVR of FVIII and FIX are normal, less amount of FIX is insufficient to normalize hemostatic parameters in comparison with FVIII.
Session topic: 33. Bleeding disorders (congenital and acquired)
Keyword(s): Hemophilia, factor VIII, Factor IX
Abstract: PB1743
Type: Publication Only
Background
Based on reports addressing hemophilia B patients bleed less common and less intensively than hemophilia A, it has been expected that the hemostatic level of factor IX (FIX) activity can be lowered than that of factor VIII (FVIII) activity.
Aims
We compared the hemostatic efficacy of the different hemostatic level of FIX and FVIII activity using global hemostatic assay.
Methods
A total of 17 severe hemophilia patients without inhibitor, aged more than 15 years old were subjected; 12 hemophilia A patients and 7 hemophilia B patients. Factor concentrates were injected to reach the target activity of 60% in hemophilia A and 40% in hemophilia B which is given by Korean health insurance guideline. All patients were in non-bleeding state and kept the wash-out period of 3 days for hemophilia A and 5 days of hemophilia B. Before and on 15 minutes after injections, we conducted one-stage factor assay, thrombin generation assay (TGA), thromboelastography (TEG) and clot-wave form analysis (CWA).
Results
Median ages of hemophilia A and hemophilia B patients were 28 and 33 years old. Baseline FVIII:C and FIX:C were 0.6% and 1.8% and they rose after injection rose to 70.8% and 49.8%. The dosage of FVIII concentrates and recombinant FIX concentrates were 28.4 IU/kg and 50.7 IU/kg. In-vivo recovery (IVR) in hemophilia A and hemophilia B patients recorded 2.43 %/U/kg and 0.91 %/U/kg. Peak thrombin of FVIII and FIX were 451.3 nM and 376.6 nM (P=0.108, normal range, 458 nM±60). TEG index of FVIII and FIX were -1.60 and -3.77 (P=0.004, normal range, -2~+2). MIN2 of CWA of FVIII and FIX were 0.62 and 0.59 (P=1.000).
Conclusion
Global hemostatic assay indicates even though IVR of FVIII and FIX are normal, less amount of FIX is insufficient to normalize hemostatic parameters in comparison with FVIII.
Session topic: 33. Bleeding disorders (congenital and acquired)
Keyword(s): Hemophilia, factor VIII, Factor IX