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In Vivo Characterization of Recombinant von Willebrand Factor in Dogs With von Willebrand Disease
Peter L. Turecek,
Herbert Gritsch,
Ludwig Pichler,
Wilfried Auer,
Bernhard Fischer,
Artur Mitterer,
Wolfgang Mundt,
Uwe Schlokat,
Friedrich Dorner,
Herm Jan M. Brinkman,
Jan A. van Mourik, and
Hans Peter Schwarz
From Immuno AG, Vienna, Austria; and The Central Laboratory of the Netherlands Red Cross Blood Transfusion Service Amsterdam, The Netherlands.
Hereditary von Willebrand factor (vWF ) deficiency in Dutch Kooiker dogs, which have undetectable levels of vWF, causes spontaneous hemorrhage of mucosal surfaces similar to the clinical picture of von Willebrand disease in humans. Therefore, we used this canine model to study the in vivo effects of a new recombinant von Willebrand factor (rvWF ) preparation containing all species of vWF multimers compared with a rvWF fraction containing only low molecular weight multimers (LMW-rvWF ) and with a plasma-derived factor VIII/vWF concentrate (pdvWF ). In the vWF-deficient dogs, the half-life of vWF:Ag was 21.6 and 22.1 hours for rvWF, 7.7 hours for pdvWF, and 9 hours for LMW-rvWF; in vivo recovery of vWF:Ag was 59%, 64%, and 70% for rvWF, 33% for pdvWF and 92% for LMW-rvWF; in vivo recovery of RCoF was 78%, 110%, and 120% for rvWF, and 25% for pdvWF. Both rvWF and pdvWF caused increases in factor VIII, which were sustained even when vWF:Ag had decreased to nearly undetectable levels and only monomeric or dimeric species were detectable on agarose gels. At the dosages used, no effect was seen on bleeding time, but the rate of blood flow from cuticle wounds was reduced after a single bolus administration of rvWF. The rvWF was able to control a severe nose bleed in one dog.
Blood, Vol. 90 No. 9 (November 1), 1997:
pp. 3555-3567
© 1997 by The American Society of Hematology.
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