BLOOD TRANSFUSION AND HEMOSTATIC ABNORMALITIES WITH SPECIAL REFERENCE TO ABNORMAL CONSTITUTION OF HEMOSTATIC PLUG IN HEMOPHILIA

The constitutional nature of hemostatic plug is important for hemostasis, because hemostatic plug might be functioning a direct role to cease bleeding at the site of hemorrhage. Therefore, the structual abnormalities of the plug in patients with hemophilia have been reported recently by us and others. However, there are many unknown mechanisms to produce the qualitative abnormalities of plug structure, which might be constructed mainly fibrin-closslinked mass and appregated platelets. In this paper, the authors observed the time course on the release of factor XIII a subunit from the platelet aggregates to the factor XIII deficient plasma by the use of our ELISA technique with polyclonal antibody against factor XIII a subunit, and it was shown a positive relationship between the release phenomenon of platelet factor XIII and the autolytic destruction process of platelet aggregates, and the 50% release of factor XIII from platelet aggregates was measured about 4hrs after platelet aggregation induced by ADP or thrombin. In the experiments of hemophilia A cases the amount of crosslinked von Willebrand factor and fibronectin to fibrin clot tended to increase but the crosslinkage of α2 plasmin inhibitor to fibrin showed a decreasing tendency in the fibrin clot obtained from the recalicified patient plasma mixed with 125I-labeled α2 plasmin inhibitor. Those data might indicate the reason for rapid fibrinolysis by urokinase added on the recalicified clot from the plasma collected before and after the administration of factor VIII reparation to hemophiliacs and also the reason for low values of the maxmum amplitude in thrombelastgraphy performed on nine patients with hemophilia A. According to the results above mentioned it might be suggested that the qualitative abnormalities of crosslinked fibrin structure of the hemostatic plug in the patients with hemophilia might effect on the disfunction of the plug, but the reaction mechanisms inducing such abnormal changes of the clot structure are still not elucidated in detail. However, the prolongation of blood clotting time due to low activation of thrombin with untimely supply of platelet factor XIII might be possible as a reacting condition for this abnormal distribution of crosslinkage.