Hemostasis in patients with severe von Willebrand disease improves after normal platelet transfusion and normalizes with further correction of the plasma defect

Authors

  • Ricardo Castillo MD,

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    • 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]

  • Ginés Escolar MD,

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    • Ginés Escolar, MD, Associate Professor, Facultad de Medicina, Universidad de Barcelona; and Head, Section of the Servicio de Hemoterapia y Hemostasia, Hospital Clinico y Provincial.

  • Juan Monteagudo MD,

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    • Juan Monteagudo, MD, Staff Member, Servicio de Hemoterapia y Hemostasia, Hospital Clinico y Provincial.

  • José Aznar-Salatti MD,

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    • José Aznar-Salatti, MD, Research Assistant, Servicio de Hemoterapia y Hemostasis, Hospital Clinico y Provincial.

  • Juan Carlos Reverter MD,

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    • Juan Carlos Reverter, MD, Staff Member, Servicio de Hemoterapia y Hemostasia, Hospital Clinico y Provincial.

  • Antonio Ordinas MD

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    • Antonio Ordinas, MD, Associate Professor, Facultad de Medicina, Universidad de Barcelona, and Head, Servicio de Hemoterapia y Hemostasia Hospital Clinico y Provincial.


Abstract

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.

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