Article Abstract

Von Willebrand disease—the Nordic perspective

Authors: Timea Szanto, Riitta Lassila, Eva Funding, Pall Torfi Onundarson, Karin Strandberg, Fariba Baghaei, on behalf of the Nordic Haemophilia Coun

Abstract

Von Willebrand disease (VWD) is the most common bleeding disorder, showing a broad variation in prevalence of 0.2 to 4 cases per 100,000 inhabitants in type 2 and 0.2 to 1 in type 3 among the Nordic countries. Diagnosis and treatment of VWD in these countries primarily occur through comprehensive treatment centers by following guidelines outlined by the Nordic Haemophilia Council. Accordingly, assignment of VWD is based on comprehensive evaluation, including standardized bleeding score, traditional laboratory analysis and adoptive platelet function studies. The correct classification of VWD types is crucial in determining both the bleeding risk and clinical management, although clinical severity is not always related to biological activity of VWF. There are several developments in both diagnosis and clinical management of VWD. This review focuses on the novel aspects of VWD care in the Nordic countries, involving pure von Willebrand factor (VWF) concentrates as treatment options, as well as laboratory assay development, including the new VWF activity assays and comprehensive whole blood platelet function and global coagulation aspects.