VWF propeptide and ratios between VWF, VWF propeptide, and FVIII in the characterization of type 1 von Willebrand disease-Reference-Cited by-同舟云学术

VWF propeptide and ratios between VWF, VWF propeptide, and FVIII in the characterization of type 1 von Willebrand disease

Published:2013-03-21 Issue:12 Volume:121 Page:2336-2339
ISSN:0006-4971
Container-title:Blood
language:en
Short-container-title:

Author:

Eikenboom Jeroen¹,Federici Augusto B.²,Dirven Richard J.¹,Castaman Giancarlo³,Rodeghiero Francesco³,Budde Ulrich⁴,Schneppenheim Reinhard⁵,Batlle Javier⁶,Canciani Maria Teresa²,Goudemand Jenny⁷,Peake Ian⁸,Goodeve Anne⁸,

Affiliation:

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

2. AB Bonomi Hemophilia and Thrombosis Centre, Foundation IRCCS Ca’ Granda Maggiore Policlinico Hospital and University of Milan, Milan, Italy;

3. Hematology Department, San Bortolo Hospital, Vicenza, Italy;

4. Hämostaseology, Medilys Laborgesellschaft mbH, Hamburg, Germany;

5. University Medical Center Hamburg-Eppendorf, Department of Pediatric Hematology and Oncology, Hamburg, Germany;

6. Servicio de Hematologia y Hemoterapia, Hospital Teresa Herrera, La Coruna, Spain;

7. University of Lille, Lille, France; and

8. Haemostasis Research Group, Department of Cardiovascular Science, University of Sheffield, Sheffield, United Kingdom

Abstract

Key Points VWFpp/VWF:Ag and FVIII:C/VWF:Ag ratios define the pathophysiological mechanisms that play a role in VWD and various VWF mutations. A high VWFpp/VWF:Ag ratio indicates increased clearance of VWF and a high FVIII:C/VWF:Ag ratio decreased synthesis of VWF.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Link

http://ashpublications.org/blood/article-pdf/121/12/2336/1364714/2336.pdf

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