von Willebrand disease update: diagnostic and treatment dilemmas.
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.
Manchester Royal Infirmary, Oxford Road, Manchester, UK. paula.bolton-maggs@manchester.ac.uk
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Bolton-Maggs
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Lillicrap
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Goudemand
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Berntorp
D003029:Coagulants / Q000627:therapeutic use; D004252:DNA Mutational Analysis; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use*; D004334:Drug Administration Schedule; D005260:Female; D006490:Hemostatics / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D008969:Molecular Sequence Data; D010641:Phenotype; D011788:Quality of Life / Q000523:psychology; D016896:Treatment Outcome; D014842:von Willebrand Diseases / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D014841:von Willebrand Factor / Q000235:genetics* / Q000378:metabolism
D003029:Coagulants; D006490:Hemostatics; D014841:von Willebrand Factor; D003894:Deamino Arginine Vasopressin