[in Japanese]

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[in Japanese]

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[in Japanese]

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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.

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There are many factors which affect hemophilic patients’ quality of life (QOL), intra-articular bleeding, inhibitor, infection caused by transfusions using unheated blood products, arthropathy, and disability, but which of these has the biggest effect is not yet clear. This study aims to find a determinant of health-related QOL (HRQOL) in hemophilia. Questionnaire survey was conducted in hemophilic patients over the age of 16. The main points investigated were the subjects’ biographical data, such as age, coagulation factor levels, employment, levels of satisfaction regarding physical and mental support, activities of daily living (ADL), and HRQOL using The RAND 36-item Health Survey 2.0 Japanese version. There were 259 (37.5%) valid responses, average age was 40.9 years old. 64.5% of patients ware severe of hemophilia. 78.8% of the patients were HCV-positive, 35.5% were HIV-positive, 8.9% were Inhibitor-positive. Results of binary step-wise logistic regression analysis show that determinant of physical aspects of HRQOL was ADL, determinant of mental aspects of HRQOL was satisfaction regarding support from work places. Comprehensive intervention is necessary to improvement of ADL and vocational assistance in order to improve the hemophilia patient’s HRQOL.

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