Copyright ©The Author(s) 2016. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hematol. Aug 6, 2016; 5(3): 61-74
Published online Aug 6, 2016. doi: 10.5315/wjh.v5.i3.61
Changing insights in the diagnosis and classification of autosomal recessive and dominant von Willebrand diseases 1980-2015
Jan Jacques Michiels, Angelika Batorova, Tatiana Prigancova, Petr Smejkal, Miroslav Penka, Inge Vangenechten, Alain Gadisseur
Jan Jacques Michiels, Goodheart Institute and Foundation in Nature Medicine and Health, Blood Coagulation and Vascular Medicine Research Center, 3069 Rotterdam, The Netherlands
Jan Jacques Michiels, Blood Coagulation Research Laboratory, University Hospital Antwerp, B-2650 Edegem, Belgium
Angelika Batorova, Tatiana Prigancova, National Hemophilia Center, Department of Hematology and Blood Transfusion, Faculty of Medicine of Comenius University, University Hospital, Antolska 11, Bratislava, Slovakia
Petr Smejkal, Miroslav Penka, Department of Clinical Hematology, University Hospital and Department of Laboratory Methods, Faculty of Medicine, Masaryk University, Jihlavska 20, Brno 62500, Czech Republic
Inge Vangenechten, Alain Gadisseur, Hemostasis Research Unit, Antwerp University Hospital, 2650 Edegem, Belgium
Author contributions: Michiels JJ, Vangenechten I and Gadisseur A analysed the literature and wrote the manuscript; all authors participated in the prospective von Willebrand disease (VWD) research study to validate the European Clinical, Laboratory and Molecular classification of VWD.
Conflict-of-interest statement: The authors declare no conflicts of interest.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Correspondence to: Jan Jacques Michiels, MD, PhD, Investigator, Senior Internist, Goodheart Institute and Foundation in Nature Medicine and Health, Blood Coagulation and Vascular Medicine Research Center, Erasmus Tower, Veenmos 13, 3069 Rotterdam, The Netherlands.
Telephone: +31-62-6970534
Received: November 26, 2015
Peer-review started: November 30, 2015
First decision: January 15, 2016
Revised: March 29, 2016
Accepted: April 14, 2016
Article in press: April 18, 2016
Published online: August 6, 2016
Core Tip

Core tip: The European Clinical Laboratory and Molecular criteria define at least 10 distinct phenotypes of von Willebrand diseases (VWD) that have significant therapeutic implications. High quality von Willebrand factor (VWF) multimeric analysis and responses to desmopressin of FVIII:C and VWF parameters are of critical diagnostic importance to document the contribution of VWF secretion, clearance, proteolysis and multimerization defects to real life phenotyping of each individual VWD patient.