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von Willebrand disease update: diagnostic and treatment dilemmas
Author(s) -
BOLTONMAGGS P. H. B.,
LILLICRAP D.,
GOUDEMAND J.,
BERNTORP E.
Publication year - 2008
Publication title -
haemophilia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.213
H-Index - 92
eISSN - 1365-2516
pISSN - 1351-8216
DOI - 10.1111/j.1365-2516.2008.01713.x
Subject(s) - von willebrand disease , medicine , von willebrand factor , disease , intensive care medicine , clinical phenotype , pediatrics , immunology , phenotype , genetics , platelet , biology , gene
Summary. 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.