Ricardo Castillo, MD, Professor of Medicine, Facultad de Medicina, Universidad de Barcelona; and Consulting Professor, Servicio de Hemoterapia y Hemostasia, Hospital Clinico y Provincial, Villaroel 170, Barcelona 08036, Spain. [Reprint requests]
Hemostasis in patients with severe von Willebrand disease improves after normal platelet transfusion and normalizes with further correction of the plasma defect
Article first published online: 27 FEB 2003
Volume 37, Issue 8, pages 785–790, August 1997
How to Cite
Castillo, R., Escolar, G., Monteagudo, J., Aznar-Salatti, J., Reverter, J. C. and Ordinas, A. (1997), Hemostasis in patients with severe von Willebrand disease improves after normal platelet transfusion and normalizes with further correction of the plasma defect. Transfusion, 37: 785–790. doi: 10.1046/j.1537-2995.1997.37897424399.x
- Issue published online: 27 FEB 2003
- Article first published online: 27 FEB 2003
- Received for publication April 17, 1996; revision received January 10, 1997, and accepted February 12, 1997.
BACKGROUND: A defective hemostatic effect of plasma concentrate infusion in patients with severe von Willebrand disease (vWD) has been ascribed to the absence of platelet von Willebrand factor (vWF) STUDY DESIGN AND METHODS: The role of platelet vWF in hemostasis of severe vWD was investigated. A plateletpheresis unit (4-5 × 10(11) platelets) from a normal compatible donor was transfused before any cryoprecipitate infusion to three type 3 vWD patients and to one patient with severe type 1 vWD with low levels of platelet vWF who required replacement therapy for bleeding episodes. Autologous platelets were transfused to one of the patients with type 3 vWD. RESULTS: Partial corrections of bleeding times (14-17 min vs. baseline>30 min) were observed in all patients after the transfusion of normal platelets. During cryoprecipitate infusion, bleeding times were normalized (<6 min), and bleeding episodes stopped when plasma levels of vWF activity ranged from 14 to 18 U per dL. Platelet interactions with the subendothelium increased in parallel with the correction of bleeding times. These results indicate that if approximately 20 percent of the total number of platelets have normal vWF antigen and if plasma vWF levels are at least 14 U per dL, then bleeding times will normalize and mucosal hemorrhages will stop. Transfusion of autologous platelets in one patient with type 3 vWD did not modify bleeding times or platelet adhesion on the subendothelium. CONCLUSION: The hemostatic effect of normal platelets in type 3 vWD seems to be related to the platelet vWF in the transfused platelets.