Haemophilia (2015), 21, 407–410

DOI: 10.1111/hae.12725

EDITORIAL

Why and how do we classify von Willebrand disease? Von Willebrand disease (VWD) is a bleeding disorder that results from inherited defects in the concentration, structure or function of the large and complex glycoprotein von Willebrand factor (VWF). As such, VWD is classified according to whether the predominant defect is a partial quantitative deficiency (type 1), qualitative defect (type 2) or complete deficiency of VWF (type 3). The secondary classification of type 2 VWD defines variants according to whether there is selective loss of high molecular weight VWF multimers (type 2A), increased or reduced binding to platelet glycoprotein Ib (types 2B and 2M respectively), or reduced binding to factor VIII (FVIII; type 2N). Yet data from the UK National Haemophilia Database show that of the 9000 patients registered with VWD, over half are not currently classified [1]. Comparable findings are reported on registries from other countries. The challenge of defining VWD type is illustrated by the patient presenting with significant bleeding symptoms in whom investigation shows reduced plasma VWF antigen levels of 0.10 IU mL 1, ristocetin cofactor binding activity of

Why and how do we classify von Willebrand disease?

Why and how do we classify von Willebrand disease? - PDF Download Free
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