抄録
The present paper is concerned with a case of von Willebrand's disease with particular reference to effect of blood transfusion on hemostatic defects.
The patient was a 10-year-old girl who had repeated nasal bleeding from early childhood, but had no hemarthrosis and gastrointestinal bleeding. Her parents were of consanguinous marriage, and her siblings had no history of bleeding tendency. Her maternal grandfather had frequent epistaxis and died of bleeding after surgical operation.
In physical examination she had no abnormalities. Examination of the peripheral blood showed a slight anemia without any significant change in count and function of leukocytes as well as blood platelets. Coagulation tests disclosed prolonged bleeding time, PTT as well as impairment of thromboplastin generating activity. Clotting time resulted in normal range. AHG level was highly reduced.
As to the treatment of this case, the transfusion of 200 ml fresh blood inproved the prolonged bleeding time and impairment of thromboplastin generating activity, and the effects continued for about 48 hours after transfusion.
