臨床研究
先天性プラスミノーゲン異常症の1家系における凝血学的検討
—特に血栓傾向の発現との関連性について—
1986 年 27 巻 2 号 p. 133-139
詳細
1986 年 27 巻 2 号 p. 133-139
In order to assess the association of thrombotic tendency with congenital plasminogen abnormality, the blood coagulation and fibrinolytic systems were investigated in 6 cases of a family with congenital plasminogen abnormality, including 4 heterozygotes and 2 homozygotes. The propositus was a heterozygote of plasminogen abnormality with homocystinuria who had been suffering from recurrent thrombosis over the past 15 years. The other affected family members were free from history of thrombosis.
The coagulation analysis of the propositus revealed mild increase of factor VIII and XII procoagulant activities, slight decrease of antithrombin III activity, moderate increase of factor VIII-related antigen and factor VIII-related ristocetin cofactor activity, and marked decrease of 6-keto-prostaglandin F1α. On the other hand, no abnormalities were found in the other affected family members, especially in 2 homozygous cases.
These results suggested that the propositus had an apparent thrombotic tendency probably resulted from homocystine-induced endothelial injury with homocystinuria. Therefore, it is postulated that the thrombosis in patients with plasminogen abnormality can occur when another additional thrombophilic factor is associated.
