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Blood, 6 August 2009, Vol. 114, No. 6, pp. 1158-1165. Prepublished online as a Blood First Edition Paper on May 27, 2009; DOI 10.1182/blood-2009-01-153296. This Article Full Text Full Text (PDF) All Versions of this Article: blood-2009-01-153296v1 114/6/1158    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via CrossRef Google Scholar Articles by Rodeghiero, F. Articles by Tosetto, A. PubMed PubMed Citation Articles by Rodeghiero, F. Articles by Tosetto, A. Related Collections How I Treat Free Research Articles Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  HOW I TREAT How I treat von Willebrand disease Francesco Rodeghiero1, Giancarlo Castaman1, and Alberto Tosetto1 1 Department of Cell Therapy and Hematology, San Bortolo Hospital, Vicenza, Italy 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. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

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