Type 1 von Willebrand disease: application of emerging data to clinical practice.
There has been much recent data published on type 1 von Willebrand disease (VWD) predominantly from three multi-centre cohort studies. These data have influenced a revision of the classification of type 1 VWD and have important implications for the management of this disorder. Patients with low von Willebrand factor (VWF) levels tend to have VWF mutations and VWD is transmitted predictably within families. In patients with VWF levels close to the lower end of the normal range, candidate mutations are found less often, ABO blood group is a more important factor and the disease has variable heritability within families. The importance of bleeding symptoms, in addition to VWF levels, in the diagnosis of type 1 VWD has been highlighted.
Arthur Bloom Haemophilia Centre, School of Medicine, Cardiff University, University Hospital of Wales, Cardiff, UK. peter.collins@cardiffandvale.wales.nhs.uk
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Collins
A M
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Cumming
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Goodeve
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Lillicrap
D000017:ABO Blood-Group System; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use; D008040:Genetic Linkage; D006490:Hemostatics / Q000627:therapeutic use; D006801:Humans; D009154:Mutation; D016896:Treatment Outcome; D014842:von Willebrand Diseases / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics; D014841:von Willebrand Factor / Q000235:genetics / Q000378:metabolism
D000017:ABO Blood-Group System; D006490:Hemostatics; D014841:von Willebrand Factor; D003894:Deamino Arginine Vasopressin