抄録
A surgical case was described of a 17-year-old female with Type I of von Willebrand's disease suffering from a chronic cystic hematoma on the left thigh. The cystic hematoma was developed during these 2 years without direct traumatic episodes and attained to a fist size. The hemostatic tests revealed that Duke bleeding time 31/2min., factor VIII procoagulant activity (VIII:C) 34%, factor VIII related antigen (VIIIR:AG) 25% and ristocetin cofactor activity (VIIIR:RC) 16%. Immediately before surgery, she was given DDAVP (0.4μg/kg) and tranexamic acid (0.75g a day). One hour after DDAVP infusion, levels of VIII:C, VIIIR:AG and VIIIR:RC increased to 78, 40 and 48%, respectively. These values returned to the preinfusion levels after 12 hours. Further infusions of DDAVP were given every 12 hours for 2 days and every 24 hours for the following 3 days. After each infusion of DDAVP, there was 2-3 fold increase of factor VIII-related activities.
The operation and the early postoperative course went on uneventfully, though a coin-sized ecchymosis appeared around the operation wound on 5th postoperative day. On the 9th postoperative day, the removal of the sutures was performed by an additional dose DDAVP without abnormal bleeding. During the course of DDAVP infusions, these was no significant side effect except a transient facial flushing.
