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Blood, 15 April 2008, Vol. 111, No. 8, pp. 3998-4003. Prepublished online as a Blood First Edition Paper on January 10, 2008; DOI 10.1182/blood-2007-08-105940. This Article Full Text Full Text (PDF) All Versions of this Article: blood-2007-08-105940v1 111/8/3998    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 HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Tosetto, A. Articles by Rodeghiero, F. Search for Related Content PubMed PubMed Citation Articles by Tosetto, A. Articles by Rodeghiero, F. Related Collections Hemostasis, Thrombosis, and Vascular Biology Free Research Articles Clinical Trials and Observations Related Article in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  CLINICAL TRIALS AND OBSERVATIONS Evidence-based diagnosis of type 1 von Willebrand disease: a Bayes theorem approach Alberto Tosetto1, Giancarlo Castaman1, and Francesco Rodeghiero1 1 Hematology Department, San Bortolo Hospital, Vicenza, Italy The diagnosis of type 1 von Willebrand disease (VWD) is based on the presence of bleeding symptoms, reduced von Willebrand factor (VWF) levels, and autosomal inheritance of the phenotype. To better appreciate the contribution of clinical and laboratory data to the final diagnosis of VWD, we computed the likelihoods of having VWD as a function of the bleeding score (LRscore), of VWF level (LRVWF), and of number of first-degree family members with reduced VWF levels (LRfamily). The 3 likelihoods were therefore combined using the Bayes theorem, giving the final probability (odds) of having VWD. LRfamily and LRVWF were the 2 factors mostly influencing the final probability of having VWD. Data from the present study provide an evidence-based description of the minimal criteria for the diagnosis of type 1 VWD. As an example, presence of VWF levels lower than 40 IU/dL in at least 2 family members (including the proband) and a bleeding score of at least 1 were found to be required for a final odd of VWD higher than 2.0 (false-positive rate less than one-half). Validation of this approach and of its clinical utility is, however, required by analysis in other cohorts of well-characterized type 1 VWD patients. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Article in Blood Online: VWD type 1: a calculated diagnosis Diana Abbott and Jorge Di Paola Blood 2008 111: 3919-3920. [Full Text] [PDF] This article has been cited by other articles: F. Rodeghiero, G. Castaman, and A. Tosetto How I treat von Willebrand disease Blood, August 6, 2009; 114(6): 1158 - 1165. [Abstract] [Full Text] [PDF] K. Ogata, M. G. Della Porta, L. Malcovati, C. Picone, N. Yokose, A. Matsuda, T. Yamashita, H. Tamura, J. Tsukada, and K. Dan Diagnostic utility of flow cytometry in low-grade myelodysplastic syndromes: a prospective validation study Haematologica, August 1, 2009; 94(8): 1066 - 1074. [Abstract] [Full Text] [PDF] A. B Federici and M. T. Canciani Clinical and laboratory versus molecular markers for a correct classification of von Willebrand disease Haematologica, May 1, 2009; 94(5): 610 - 615. [Full Text] [PDF] J. E. Sadler Low von Willebrand factor: sometimes a risk factor and sometimes a disease Hematology, January 1, 2009; 2009(1): 106 - 112. [Abstract] [Full Text] [PDF] F. Rodeghiero, G. Castaman, and A. Tosetto Optimizing treatment of von Willebrand disease by using phenotypic and molecular data Hematology, January 1, 2009; 2009(1): 113 - 123. [Abstract] [Full Text] [PDF]

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