von Willebrand disease update: diagnostic and treatment dilemmas

P H B Bolton-Maggs1, D Lillicrap, J Goudemand

  • 1Manchester Royal Infirmary, Oxford Road, Manchester, UK. paula.bolton-maggs@manchester.ac.uk

Although von Willebrand disease (VWD) is now well-described, many facets of diagnosis and management continue to be debated. The diagnosis of type 1 disease can be difficult but recent genetic analyses help to distinguish many factors which can influence von Willebrand factor (VWF) levels and bleeding phenotype. Type 2 disease (functional abnormalities) includes a particularly interesting group of disorders with faulty binding between VWF and FVIIIC (Normandy) where treatment methods need careful consideration. Type 3 VWD is the most severe form of VWD and a new international study is underway to examine the use of prophylaxis.

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