von Willebrand factor propeptide and the phenotypic classification of von Willebrand disease

Author:

Sanders Yvonne V.1,Groeneveld Dafna2,Meijer Karina3,Fijnvandraat Karin4,Cnossen Marjon H.5,van der Bom Johanna G.67,Coppens M.8,de Meris Joke9,Laros-van Gorkom Britta A. P.10,Mauser-Bunschoten Eveline P.11,Leebeek Frank W. G.1,Eikenboom Jeroen2,

Affiliation:

1. Hematology, Erasmus University Medical Center Rotterdam, Rotterdam, The Netherlands;

2. Einthoven Laboratory for Experimental Vascular Medicine, Department of Thrombosis and Hemostasis, Leiden University Medical Center, Leiden, The Netherlands;

3. University of Groningen, Department of Hematology, University Medical Center Groningen, Groningen, The Netherlands;

4. Pediatric Hematology, Emma Children’s Hospital, Academic Medical Center, Amsterdam, The Netherlands;

5. Pediatric Hematology, Erasmus University Medical Center-Sophia Children’s Hospital Rotterdam, Rotterdam, The Netherlands;

6. Clinical Epidemiology, Leiden University Medical Center, Leiden, The Netherlands;

7. Jon J. van Rood Center for Clinical Transfusion Medicine, Sanquin Research, Leiden, The Netherlands;

8. Vascular Medicine, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands;

9. Netherlands Hemophilia Society, Nijkerk, The Netherlands;

10. Hematology, Radboud University Medical Center, Nijmegen, The Netherlands; and

11. Van Creveldkliniek/Hematology, University Medical Center Utrecht, Utrecht, The Netherlands

Abstract

Key Points VWFpp discriminates between type 3 VWD patients and severe type 1 VWD patients with very low VWF levels. The pathophysiological mechanisms of all types of VWD can be defined by the combined ratios of VWFpp/VWF:Ag and FVIII:C/VWF:Ag.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

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