Advances in the diagnosis and classification of von Willebrand disease

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Von Willebrand disease, the most frequently inherited bleeding disorder, has a very varied phenotype, which has made classification difficult since its discovery. Several classification systems have been put forward over the years by experts and international organizations like the ISTH, often coming short of satisfactorily incorporating all the different types. Advances in routine laboratory techniques, multimeric analysis and molecular studies have led to more questions than answers about classifying von Willebrand disease. In this publication, the authors have tried to address the value of current diagnostic techniques, many of them only available to specialized centers, in order to come to a classification proposal which can comfortably accommodate all different types of von Willebrand disease. New developments such as the von Willebrand Propeptide analysis have been included as well as the diagnostic importance of DDAVP testing. This publication is indispensable to investigators of this bleeding disorder and to clinicians and researchers searching for a deeper understanding of this disease.