{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,7]],"date-time":"2025-11-07T09:14:05Z","timestamp":1762506845863},"reference-count":40,"publisher":"Wiley","issue":"S1","license":[{"start":{"date-parts":[[2016,7,17]],"date-time":"2016-07-17T00:00:00Z","timestamp":1468713600000},"content-version":"vor","delay-in-days":77,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2015,9,1]],"date-time":"2015-09-01T00:00:00Z","timestamp":1441065600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["wiley.com"],"crossmark-restriction":true},"short-container-title":["Int J Lab Hematology"],"published-print":{"date-parts":[[2016,5]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>von Willebrand disease (<jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content>) is the most common inherited bleeding disorder and is due to a deficiency and\/or abnormality of von Willebrand factor (<jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>). <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content> is inherited in an autosomal dominant or recessive pattern, but women are apparently more symptomatic. Diagnosis of <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content> is still difficult in most countries due to the multiple activities of <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content> and the heterogeneity of the disease. <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content> is mainly associated with mild mucosal bleeding although gastrointestinal and joint bleeds may occur in severe <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content> forms. This review describes the most recent clinical and laboratory procedures for the correct diagnosis of <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content>. Assays for the evaluation of the platelet\u2010dependent <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content> activity (<jats:styled-content style=\"fixed-case\">PD<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>act) with or without ristocetin as well as <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content> collagen binding (<jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>:<jats:styled-content style=\"fixed-case\">CB<\/jats:styled-content>) are currently in use. However, other tests such as <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content> antigen (<jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>:Ag), factor <jats:styled-content style=\"fixed-case\">VIII<\/jats:styled-content> procoagulant (<jats:styled-content style=\"fixed-case\">FVIII<\/jats:styled-content>:C), ristocetin\u2010induced platelet agglutination (<jats:styled-content style=\"fixed-case\">RIPA<\/jats:styled-content>), multimeric analysis (<jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>:<jats:styled-content style=\"fixed-case\">MA<\/jats:styled-content>), <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content> propeptide (<jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>pp), <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>:<jats:styled-content style=\"fixed-case\">FVIII<\/jats:styled-content> binding assay (<jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>:<jats:styled-content style=\"fixed-case\">FVIIIB<\/jats:styled-content>), and the assessment of biological response to desmopressin (<jats:styled-content style=\"fixed-case\">DDAVP<\/jats:styled-content>) are necessary to characterize <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content> types. Levels of <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content> activities &lt;30\u00a0U\/<jats:styled-content style=\"fixed-case\">dL<\/jats:styled-content> have been associated with a bleeding phenotype and the presence of mutations in the <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content> gene.<\/jats:p>","DOI":"10.1111\/ijlh.12540","type":"journal-article","created":{"date-parts":[[2016,7,18]],"date-time":"2016-07-18T03:40:25Z","timestamp":1468813225000},"page":"41-49","update-policy":"http:\/\/dx.doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Current and emerging approaches for assessing von Willebrand disease in 2016"],"prefix":"10.1111","volume":"38","author":[{"given":"A. B.","family":"Federici","sequence":"first","affiliation":[{"name":"Hematology and Transfusion Medicine Department of Oncology and Onco\u2010Hematology L. 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