Congenital bleeding disorders with long bleeding time and normal platelet count. II. Von Willebrand's disease (report of thirty-seven patients).

摘要: Abstract The hemostatic data on thirty-seven patients with von Willebrand's disease are reported. diagnostic criteria included prolonged bleeding time, low platelet adhesiveness to glass beads and factor VIII deficiency. This study of our supports the view that is heterogeneous, since time may be normal VII level very variable in some patients. behavior platelet-rich plasma presence concentrations adenosine diphosphate (ADP) was found abnormal except two families. Platelet consistently abnormal. dominant autosomal mode inheritance, due a pleiotropic gene, expressed variety ways, as illustrated by relatives this series. In vivo experiments confirm existence an underlying plasmatic defect deficiency corrected infusion or hemophilic platelet-poor plasma. same biochemical responsible for both anomalies, although "Willebrand factor" precursor remain unidentified.