52 Effect of Diabetic Plasma in von Willebrand's Disease

Abstract

Although in vivo correction of antihemophilic factor (AHF) levels is easily achieved in von Willebrand's disease (vWd), correction of the bleeding time (BT) defect is difficult by usual transfusion therapy. In an effort to find more effective treatment for four children with severe vWd (low AHF levels, prolonged BT, defective platelet adhesiveness), transfusion studies were done. Plasma AHF levels, BT's (modified Ivy), and native blood platelet adhesiveness tests were done before and after transfusions of fresh and fresh-frozen platelet-free ACD plasma. The results showed that plasma obtained from donors with diabetes mellitus (juvenile onset) corrected the bleeding time and platelet adhesiveness test temporarily in all four patients when doses of 10–15 ml/kg were used. Comparable dosages of normal plasma were effective in correcting the BT in only one patient. AHF-rich fibrinogen, cryoprecipitates of normal plasma, and plasma from an exercised donor did not correct the BT. The effectiveness of the diabetic plasma was approximately directly proportional to the severity of the diabetes. Also, the immediate rise in AHF levels was greater following diabetic plasma infusion than after normal plasma. Mixtures of vWd blood and fractions of plasma were tested for platelet adhesiveness (PA) by the in vitro method of HELLEM. Diabetic plasma and cryoprecipitate showed excellent correction of the defective PA; AHF-fibrinogen and normal plasma showed moderate correction, and diabetic and normal serum, normal cryoprecipitate, and dextrose showed poor PA. These in vitro and in vivo studies suggest that the blood from certain diabetics contains an increased amount of the factor (s) responsible for correction of the bleeding time and platelet adhesiveness defect found in vWd. (SPR)