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von Willebrand’s disease diagnosis and laboratory issues
Author(s) -
CASTAMAN G.,
MONTGOMERY R. R.,
MESCHENGIESER S. S.,
HABERICHTER S. L.,
WOODS A. I.,
LAZZARI M. A.
Publication year - 2010
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.1365-2516.2010.02296.x
Subject(s) - medicine , von willebrand disease , disease , intensive care medicine , von willebrand factor , pathology , immunology , platelet
Summary. In this paper, the recent developments in the diagnosis and laboratory issues of von Willebrand’s disease (VWD) are presented. Dr. Castaman reviews the functional tests available for the diagnosis of VWD and their pathophysiological significance, focusing on which tests are best used in the diagnosis and classification of VWD. Dr Montgomery reviews an emerging issue that is accelerated clearance of von Willebrand factor (VWF) occurring in some variants of VWD. This phenotype can be suspected by the presence of an increased ratio between the VWF propeptide and the VWF antigen. These patients have typically a robust, but short‐lived increase of FVIII and VWF after desmopressin. Dr Meschengieser reviews the determinants of bleeding after surgery in patients with VWD, emphasizing the role of bleeding history in predicting this risk.