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• W2093578295 abstract "Abstract Recent multicenter studies have clarified the molecular basis underlying the different von Willebrand disease (VWD) types, all of which are caused by the deficiency and/or abnormality of von Willebrand factor (VWF). These studies have suggested a unifying pathophysiologic concept. The diagnosis of VWD, remains difficult because its clinical and laboratory phenotype is very heterogeneous and may overlap with normal subjects. Stringent criteria are therefore required for a clinically useful diagnosis. In this paper, we delineate a practical approach to the diagnosis and treatment of VWD. Our approach is based on the critical importance of a standardized bleeding history that has been condensed into a final bleeding score and a few widely available laboratory tests, such as VWF ristocetin cofactor activity, VWF antigen and factor VIII. This approach would help identify those subjects who will probably benefit from a diagnosis of VWD. The next step involves performing a trial infusion with desmopressin in all patients who fail to exhibit an enhanced responsiveness to ristocetin. On the basis of these results and through a series of illustrative examples, the clinician will be able to select the best approach for the optimal management of VWD, according to the patient's characteristics and clinical circumstances." @default.
• W2093578295 created "2016-06-24" @default.
• W2093578295 creator A5006881585 @default.
• W2093578295 creator A5013529815 @default.
• W2093578295 creator A5047717272 @default.
• W2093578295 date "2009-08-06" @default.
• W2093578295 modified "2023-09-30" @default.
• W2093578295 title "How I treat von Willebrand disease" @default.
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• W2093578295 doi "https://doi.org/10.1182/blood-2009-01-153296" @default.
• W2093578295 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/19474451" @default.
• W2093578295 hasPublicationYear "2009" @default.
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