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 Blood, 15 May 2008, Vol. 111, No. 10, pp. 4979-4985.
Prepublished online as a Blood First Edition Paper on March 14, 2008; DOI 10.1182/blood-2007-09-110940.

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HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY

Identification of type 1 von Willebrand disease patients with reduced von Willebrand factor survival by assay of the VWF propeptide in the European study: Molecular and Clinical Markers for the Diagnosis and Management of Type 1 VWD (MCMDM-1VWD)

Sandra L. Haberichter13, Giancarlo Castaman4, Ulrich Budde5, Ian Peake6, Anne Goodeve6, Francesco Rodeghiero4, Augusto B. Federici7, Javier Batlle8, Dominique Meyer9, Claudine Mazurier10, Jenny Goudemand11, Jeroen Eikenboom12, Reinhard Schneppenheim13, Jorgen Ingerslev14, Zdena Vorlova15, David Habart15, Lars Holmberg16, Stefan Lethagen16, John Pasi17, Frank G. H. Hill18, and Robert R. Montgomery13

1 Department of Pediatrics, Medical College of Wisconsin, Milwaukee; 2 Children's Research Institute, Children's Hospital of Wisconsin, Milwaukee; 3 Blood Research Institute, BloodCenter of Wisconsin, Milwaukee; 4 San Bortolo Hospital, Vicenza, Italy; 5 Coagulation Laboratory, Hamburg, Germany; 6 University of Sheffield, Sheffield, United Kingdom; 7 Istituti di ricovero e cura a carattere scientifico (IRCCS) Foundation Maggiore Hospital, University of Milan, Milan, Italy; 8 Hospital Teresa Herrera, La Coruna, Spain; 9 Inserm, Paris, France; 10 Laboratoire Français du Fractionnement et des Biotechnologies, Lille, France; 11 University of Lille, Lille, France; 12 Leiden University Medical Center, Leiden, The Netherlands; 13 University Medical Center Hamburg-Eppendorf, Department of Pediatric Hematology and Oncology, Hamburg, Germany; 14 University Hospital Skejby, Aarhus, Denmark; 15 Institute of Hematology and Blood Transfusion, Prague, Czech Republic; 16 University of Lund, Malmo, Sweden; 17 Barts and The London, Queen Mary University of London, London, United Kingdom; and 18 Children's Hospital, Birmingham, United Kingdom

The decreased survival of von Willebrand factor (VWF) in plasma has been implicated as a mechanism in a subset of type 1 von Willebrand disease (VWD) patients. We have previously reported that the ratio of plasma levels of VWF and its propeptide (VWFpp) can be used to identify patients with reduced VWF survival. In this study, we report the assay of VWFpp and VWF:Ag in 19 individuals recruited from 6 European centers within the MCMDM-1VWD study. Eight individuals had a VWF:Ag level less than 30 IU/dL. Seven of these patients had a robust desmopressin response and significantly reduced VWF half-life that was predicted by a markedly increased steady-state plasma VWFpp/VWF:Ag ratio. VWF mutations previously associated with reduced VWF survival were identified in each of the 7 individuals. Thus, a substantially increased ratio of steady-state VWFpp/VWF:Ag predicted a reduced VWF half-life in patients with markedly decreased VWF:Ag levels. These data indicate that a reduced VWF survival is found in a subpopulation of patients with type 1 VWD. The systematic assay of both plasma VWF and the VWF propeptide in moderately severe type 1 VWD patients may identify patients with a reduced VWF survival phenotype.


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