{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,26]],"date-time":"2026-03-26T10:20:45Z","timestamp":1774520445670,"version":"3.50.1"},"reference-count":50,"publisher":"Georg Thieme Verlag KG","issue":"06","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Semin Thromb Hemost"],"published-print":{"date-parts":[[2022,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>von Willebrand disease (VWD) is reportedly the most common inherited bleeding disorder, and can also arise as an acquired (von Willebrand) syndrome (AVWS). The hemostasis laboratory plays a key role in the diagnosis or exclusion of VWD\/AVWS, which may otherwise be suspected due to the patient's clinical (bleeding) history. VWD\/AVWS arise from deficiency and\/or defects in the adhesive plasma protein, von Willebrand factor (VWF). VWF undertakes various roles within hemostasis, but principally acts within primary hemostasis to anchor platelets to sites of vascular damage, thereby facilitating thrombus formation to arrest bleeding. The diagnosis or exclusion of VWD\/AVWS requires laboratory testing for both VWF level and activity, with the latter potentially comprising several of a potential plethora of different assays. Complete diagnosis of VWD also requires the differentiation of VWD type, with six types comprising the current classification (i.e., qualitative [types 2A, 2B, 2M, 2N VWD] vs. quantitative [types 1 and 3 VWD] deficiency\/defects). Although appropriate diagnosis and type classification hold important therapeutic consequences, these remain problematic and sometimes elusive for some laboratories to achieve. This report reviews the laboratory aided diagnosis or exclusion of VWD from a geographic perspective, and focuses on the disparities of approaches and methods in different regions of the world. This is primarily done from the perspective of data available from published reports related to external quality assessment (or proficiency testing) from different geographic localities. Moreover, differences in approaches between laboratories may arise due to differential adherence of particular guidelines, as well as regulatory aspects and predominance of local manufacturers and suppliers.<\/jats:p>","DOI":"10.1055\/s-0042-1754331","type":"journal-article","created":{"date-parts":[[2022,9,2]],"date-time":"2022-09-02T22:50:01Z","timestamp":1662159001000},"page":"750-766","source":"Crossref","is-referenced-by-count":13,"title":["Laboratory Diagnosis of von Willebrand Disease (VWD): Geographical Perspectives"],"prefix":"10.1055","volume":"48","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2103-1661","authenticated-orcid":false,"given":"Emmanuel J.","family":"Favaloro","sequence":"additional","affiliation":[{"name":"Department of Haematology, Institute of Clinical Pathology and Medical Research (ICPMR), NSW Health Pathology, Westmead Hospital, Westmead, North South Wales, Australia"},{"name":"Sydney Centres for Thrombosis and Haemostasis, Westmead, North South Wales, Australia"},{"name":"Faculty of Science and Health, Charles Sturt University, Wagga Wagga, North South Wales, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8237-6743","authenticated-orcid":false,"given":"Leonardo","family":"Pasalic","sequence":"additional","affiliation":[{"name":"Department of Haematology, Institute of Clinical Pathology and Medical Research (ICPMR), NSW Health Pathology, Westmead Hospital, Westmead, North South Wales, Australia"},{"name":"Sydney Centres for Thrombosis and Haemostasis, Westmead, North South Wales, Australia"},{"name":"Westmead Clinical School, University of Sydney, Westmead, North South Wales, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2022,9,2]]},"reference":[{"issue":"05","key":"ref1","doi-asserted-by":"crossref","first-page":"440","DOI":"10.1055\/s-0031-1281028","article-title":"Von Willebrand disease: local diagnosis and management of a globally distributed bleeding disorder","volume":"37","author":"E J Favaloro","year":"2011","journal-title":"Semin Thromb Hemost"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"755","DOI":"10.2147\/JBM.S266791","article-title":"Why is misdiagnosis of von Willebrand disease still prevalent and how can we overcome it? A focus on clinical considerations and recommendations","volume":"12","author":"C K Colonne","year":"2021","journal-title":"J Blood Med"},{"issue":"01","key":"ref3","first-page":"17","article-title":"Von Willebrand factor: form for function","volume":"40","author":"A Yee","year":"2014","journal-title":"Semin Thromb Hemost"},{"issue":"04","key":"ref4","doi-asserted-by":"crossref","first-page":"431","DOI":"10.1055\/s-0040-1713735","article-title":"Navigating the myriad of von Willebrand factor assays","volume":"40","author":"E J Favaloro","year":"2020","journal-title":"Hamostaseologie"},{"issue":"10","key":"ref5","doi-asserted-by":"crossref","first-page":"2103","DOI":"10.1111\/j.1538-7836.2006.02146.x","article-title":"Update on the pathophysiology and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand Factor","volume":"4","author":"J E Sadler","year":"2006","journal-title":"J Thromb 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