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[Capacity of activation of so-called deficient factors in hereditary blood coagulation disorders]
By factor-specific exchange-experiments it was proved that the lack of factor VIII activity in hemophilia A is not caused by an incapacity of activation or even lack of the factor, but by a defect of the pertaining activator resp. activator system. The same goes for factor IX in hemophilia B as well as factors VII, IX and X in family defect of the prothrombin-complex. In contrast, inactivity in the von Willebrand-Jünrgens-disease is obviously caused by a genuine lack of factor VIII.
By factor-specific exchange-experiments it was proved that the lack of factor VIII activity in hemophilia A is not caused by an incapacity of activation or even lack of the factor, but by a defect of the pertaining activator resp. activator system. The same goes for factor IX in hemophilia B as well as factors VII, IX and X in family defect of the prothrombin-complex. In contrast, inactivity in the von Willebrand-Jünrgens-disease is obviously caused by a genuine lack of factor VIII.