Abstract
Hemophilia can be generally classified into three groups, severe (<1 IU/dl), moderate (1–5 IU/dl), and mild type (5≪40 IU/dl), since factor VIII (or IX) activity highly correlates with the clinical severity. The heterogeneity in clinical severity of their patients has been recognized, however. Some factors can be raised in the divergence of clinical phenotype and factor VIII (or IX) activity, and there are limitations to the concept that their activities obtained in one-stage clotting assay reflect the whole hemostatic coagulation in vivo. In recent years, the evaluation for the dynamic and comprehensive blood clotting function is of current interest, representative thromboelastography, clot waveform analysis, and thrombin generation test, in addition total thrombus-formation analysis system. Evaluation of qualitative parameters in the coagulation process is possible. Therefore, the combination with dynamic and comprehensive coagulation function and factor VIII (or IX) activity could provide the potential ability of hemostatic coagulation of the patients. In addition, it would be useful for the prediction of bleeding severity and the designation of adequate and long-term hemostatic management in their lives.
