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Despite its prevalence and associated morbidity, the diagnosis and subclassification of VWD continue to pose significant clinical challenges. This is in part attributable to the fact that plasma von Willebrand factor (VWF) levels vary over a wide range in the normal population, together with the multiple different physiological functions played by VWF<jats:italic>in vivo<\/jats:italic>. Over recent years, substantial progress has been achieved in elucidating the biological roles of VWF. Significant advances have also been made into defining the pathophysiological mechanisms underpinning both quantitative and qualitative VWD. In particular, several new laboratory assays have been developed that enable more precise assessment of specific aspects of VWF activity. In the present review, we discuss these recent developments in the field of VWD diagnosis, and consider how these advances can impact upon clinical diagnostic algorithms for use in routine clinical practice. In addition, we review some important recent advances pertaining to the various treatment options available for managing patients with VWD.<\/jats:p>","DOI":"10.1111\/bjh.16681","type":"journal-article","created":{"date-parts":[[2020,5,12]],"date-time":"2020-05-12T09:03:12Z","timestamp":1589274192000},"page":"329-339","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":47,"title":["New developments in von Willebrand disease"],"prefix":"10.1111","volume":"191","author":[{"given":"Helen","family":"Fogarty","sequence":"first","affiliation":[{"name":"Irish Centre for Vascular Biology School of Pharmacy and Biomolecular Sciences Royal College of Surgeons in Ireland Dublin Ireland"},{"name":"National Coagulation Centre St James's Hospital Dublin Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dearbhla","family":"Doherty","sequence":"additional","affiliation":[{"name":"Irish Centre for Vascular Biology School of Pharmacy and Biomolecular Sciences Royal College of Surgeons in Ireland Dublin Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James S.","family":"O'Donnell","sequence":"additional","affiliation":[{"name":"Irish Centre for Vascular Biology School of Pharmacy and Biomolecular Sciences Royal College of Surgeons in Ireland Dublin Ireland"},{"name":"National Coagulation Centre St James's Hospital Dublin Ireland"},{"name":"National Children's Research Centre Our Lady's Children's Hospital Crumlin Dublin Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2020,5,12]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1182\/asheducation-2013.1.254"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1601561"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2009.03661.x"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V69.2.454.454"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0038-1647491"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0037-1613992"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1160\/TH12-04-0244"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.24195"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.blre.2012.09.002"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1160\/TH11-03-0180"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.13064"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2007.01643.x"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.13508"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/jth.12594"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1182\/asheducation-2016.1.683"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2017-05-782029"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2006.02146.x"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2012.077263"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2012.04923.x"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12747"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2012.04775.x"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2008.03182.x"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2009.03499.x"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2005.01663.x"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2010.03975.x"},{"key":"e_1_2_8_27_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2006.01847.x"},{"key":"e_1_2_8_28_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12503"},{"key":"e_1_2_8_29_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2010.03796.x"},{"key":"e_1_2_8_30_1","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2018017418"},{"key":"e_1_2_8_31_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2014-02-557264"},{"key":"e_1_2_8_32_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.transci.2018.07.005"},{"key":"e_1_2_8_33_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2257.2000.00281.x"},{"key":"e_1_2_8_34_1","first-page":"1180","article-title":"Evaluation of an automated method for the quantification of von Willebrand factor antigen. 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