The pathogenesis of thrombosis and embolic disorders has been partially clarified. This is related to patients with deficiency or abnormalities of antithrombin III, protein C (PC) and patients with aquired lupus anticoagulant (LA). However, most of the patients showed no abnormal laboratory findings, even though they had thrombosis or embolic disorders.
Recently, several reports have suggested the presense of patients with thrombophilic disposition and poor anticoagulant response to activated protein C (APC).
We have measured poor anticoagulant response to APC in healthy subjects.
Results obtained herein are as follows:
1) The data of plasma samples were affected by freezing procesures and numbers of platelets. Therefore, freshly availabie platelet poor plasms was recommended.
2) The distribution of APC ration in healthy subjects were 2.0-4.0.

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Dieulafoy's ulcer, a special category, is one of the serious gastric lesions with a high mortality rate even in individuals without bleeding tendency. We present two cases of hemophilia A with high-responder inhibitor associated with Dieulafoy's ulcer in which treatment with a combination of endoscopic hemostatic procedure and inhibitor by-passing therapy was effective.
Both patients were very pale and in acute distress on admission. Endoscopic examination of the stomach under an infusion of FEIBA^® revealed hemorrhage from an exposed artery and a diagnosis of Dieulafoy's ulcer was established. Absolute ethanol and hypertonic saline containing 0.005% epinephrine were injected into the exposed vessel and its surroundings. These procedures were performed easily and safely and local hemastasis was obtained.
This is the first reported cases of successful management of Dieulafoy's ulcer in patients with hemophilia A with inhibitor using a combination of local hemostasis with an endoscope and FEIBA^® infusion.

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Factor XI deficiency is a rare hereditary coagulation disorder and mostly its bleeding manifestation is excessive bleeding after surgery and trauma. Hemostasis of a 69-year-old Japanese woman with factor XI deficiency in total hip joint replacement was successfully achieved by plasma replacement therapy. Plasma exchange was took place preoperatively with 2800ml of fresh frozen plasma. Factor XI level of the patient plasma showed 65% of normal immediately after the plasma exchange and 57% at the operation. Subsequently, fresh frozen plasmas were transfused to maintain factor XI level at 40% for 24 hours after surgery then 30% for the next 3 days. Factor XI level and APTT were monitored and no excessive bleeding was observed during and after the operation.

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