title: Hemostatic effect of normal platelet transfusion in severe von Willebrand disease patients.
abstract: Platelet von Willebrand factor (vWF) has been suggested to play an important role in the hemostatic process. Clinical and experimental data indicate that bleeding time (BT) and platelet-vessel wall interaction cannot be normalized unless the defect of platelet vWF is also corrected. We have examined the effect of normal platelet concentrate transfusion 1 hour after cryoprecipitate infusion in five type III von Willebrand disease (vWD) patients. The cryoprecipitate infusion attained normal circulating levels of ristocetin cofactor, vWF antigen, and factor VIII activity. In two patients, cryoprecipitate infusion did not modify the BT (greater than 30 minutes), whereas in the remaining three patients BT was only partially corrected (from greater than 30 to 12, 18, and 21 minutes). However, the immediate platelet transfusion completely corrected the BT in four cases, and in one case it shortened the BT to 8.30 minutes (n = 3 to 8 minutes). In the perfusion study, cryoprecipitate infusion only resulted in a slight increase in platelet deposition (surface coverage range: 2.4% to 11.3%), whereas the platelet concentrate transfusion elicited a more marked improvement (range: 8.2% to 26.4%; P less than .02 v post-cryoprecipitate). These results suggest an important in vivo role of the platelet vWF in supporting platelet-vessel wall interaction. They also give support to the occasional addition of normal platelet transfusion to the cryoprecipitate infusion for the control of serious bleeding episodes resistant to cryoprecipitate in severe vWD patients.
authors.affiliation: Servicio de Hemoterapia y Hemostasia, Hospital Clínico y Provincial, Facultad de Medicina, Barcelona, Spain.
authors.forename: R
authors.initials: R
authors.lastname: Castillo
authors.forename: J
authors.initials: J
authors.lastname: Monteagudo
authors.forename: G
authors.initials: G
authors.lastname: Escolar
authors.forename: A
authors.initials: A
authors.lastname: Ordinas
authors.forename: M
authors.initials: M
authors.lastname: Magallón
authors.forename: J
authors.initials: J
authors.lastname: Martín Villar
mesh_terms: D001760:Bleeding Time; D001792:Blood Platelets / Q000473:pathology; D001803:Blood Transfusion; D004730:Endothelium, Vascular / Q000473:pathology; D005169:Factor VIII / Q000378:metabolism / Q000627:therapeutic use; D005340:Fibrinogen / Q000627:therapeutic use; D006487:Hemostasis; D006801:Humans; D017713:Platelet Transfusion; D012310:Ristocetin / Q000097:blood; D014842:von Willebrand Diseases / Q000097:blood / Q000473:pathology / Q000628:therapy*; D014841:von Willebrand Factor / Q000378:metabolism
chemical_list: C026912:cryoprecipitate coagulum; D014841:von Willebrand Factor; D012310:Ristocetin; D005169:Factor VIII; D005340:Fibrinogen