[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"doc-seo-448207-105":3,"detail-sidebar-cat-0-en-105":81,"doc-detail-448207-en":131},{"code":4,"msg":5,"data":6},0,"ok",{"site_id":7,"language":8,"slug":9,"title":10,"keywords":11,"description":12,"schema_data":13,"social_meta":74,"head_meta":76,"extra_data":78,"updated_unix":80},105,"en","von-willebrand-disease-classification-and-epidemiology","Von Willebrand disease: classification and epidemiology","","Von Willebrand disease (VWD) is a highly heterogeneous inherited bleeding disorder caused by reduced von Willebrand factor (VWF) levels or activity. Diagnosis relies on a positive bleeding history, low circulating VWF levels, and autosomal inheritance patterns. VWD includes quantitative deficiencies (type 1, partial; type 3, almost total) and qualitative defects (type 2A, 2B, 2M, 2N). Reported prevalence is ~1% but clinical diagnosis is complicated by confounding factors, especially in mild cases. Genomic data suggest pathogenic VWF variants may be more common.",{"@graph":14,"@context":73},[15,34,56],{"@type":16,"itemListElement":17},"BreadcrumbList",[18,23,27,31],{"item":19,"name":20,"@type":21,"position":22},"https://docshare.wps.com","Home","ListItem",1,{"item":24,"name":25,"@type":21,"position":26},"https://docshare.wps.com/document/","Document",2,{"item":28,"name":29,"@type":21,"position":30},"https://docshare.wps.com/document/healthcare/","Healthcare",3,{"item":32,"name":10,"@type":21,"position":33},"https://docshare.wps.com/document/von-willebrand-disease-classification-and-epidemiology/448207/",4,{"url":32,"name":10,"@type":35,"image":36,"author":41,"headline":10,"publisher":44,"fileFormat":47,"inLanguage":8,"description":12,"dateModified":48,"datePublished":49,"encodingFormat":47,"isAccessibleForFree":50,"interactionStatistic":51},"DigitalDocument",{"url":37,"@type":38,"width":39,"height":40},"https://docshare.wps.com/thumbnails/von-willebrand-disease-classification-and-epidemiology/448207.png","ImageObject",300,407,{"name":42,"@type":43},"Levi","Person",{"url":19,"name":45,"@type":46},"DocShare","Organization","application/pdf","2026-11-10","2026-09-29",true,{"@type":52,"interactionType":53,"userInteractionCount":55},"InteractionCounter",{"@type":54},"ViewAction",12,{"@type":57,"mainEntity":58},"FAQPage",[59,65,69],{"name":60,"@type":61,"acceptedAnswer":62},"What criteria are used to diagnose Von Willebrand disease (VWD)?","Question",{"text":63,"@type":64},"VWD diagnosis is based on three main criteria: a positive bleeding history, low levels of circulating VWF, and autosomal inheritance patterns.","Answer",{"name":66,"@type":61,"acceptedAnswer":67},"How is VWD classified by VWF abnormalities?",{"text":68,"@type":64},"VWD is classified into quantitative VWF deficiencies (type 1 and type 3) and qualitative VWF defects (type 2 subtypes A, B, M, and N).",{"name":70,"@type":61,"acceptedAnswer":71},"Why is the prevalence of clinically significant VWD difficult to determine?",{"text":72,"@type":64},"Prevalence estimates vary because they depend on clinical criteria used to select subjects, population studied, laboratory criteria for confirmation, and the challenges of assessing bleeding history in epidemiological settings.","https://schema.org",{"og:url":32,"og:type":75,"og:title":10,"og:site_name":45,"og:description":12},"article",{"robots":77,"canonical":32},"index,follow",{"doc_id":79,"site_id":7},448207,1790793874,{"code":4,"msg":82,"data":83},"success",[84,88,92,96,101,106,110,115,120,123,127],{"id":22,"doc_module":4,"doc_module_name":25,"category_name":85,"show_sort_weight":86,"slug":87},"Story & Novel",90,"story-novel",{"id":26,"doc_module":4,"doc_module_name":25,"category_name":89,"show_sort_weight":90,"slug":91},"Literature",80,"literature",{"id":33,"doc_module":4,"doc_module_name":25,"category_name":93,"show_sort_weight":94,"slug":95},"Exam",70,"exam",{"id":97,"doc_module":4,"doc_module_name":25,"category_name":98,"show_sort_weight":99,"slug":100},5,"Comic",60,"comic",{"id":102,"doc_module":4,"doc_module_name":25,"category_name":103,"show_sort_weight":104,"slug":105},6,"Technology",50,"technology",{"id":107,"doc_module":4,"doc_module_name":25,"category_name":29,"show_sort_weight":108,"slug":109},7,40,"healthcare",{"id":111,"doc_module":4,"doc_module_name":25,"category_name":112,"show_sort_weight":113,"slug":114},8,"Research & Report",30,"research-report",{"id":116,"doc_module":4,"doc_module_name":25,"category_name":117,"show_sort_weight":118,"slug":119},9,"Religion & Spirituality",20,"religion-spirituality",{"id":118,"doc_module":4,"doc_module_name":25,"category_name":121,"show_sort_weight":118,"slug":122},"World Cup","world-cup",{"id":124,"doc_module":4,"doc_module_name":25,"category_name":125,"show_sort_weight":124,"slug":126},10,"Lifestyle","lifestyle",{"id":128,"doc_module":4,"doc_module_name":25,"category_name":129,"show_sort_weight":97,"slug":130},19,"General","general",{"code":4,"msg":82,"data":132},{"doc_id":79,"user_id":133,"nickname":42,"user_avatar":134,"doc_module":4,"category_id":107,"category_name":29,"doc_title":10,"doc_description":12,"doc_content":135,"file_id":136,"file_url":137,"file_type":138,"file_size":139,"view_count":55,"is_deleted":4,"is_public":22,"is_downloadable":22,"audit_status":22,"page_count":116,"language":140,"language_code":8,"site_id":7,"html_lang":8,"table_of_contents":141,"faqs":142,"seo_title":143,"seo_description":12,"update_tm":144,"read_time":145,"preview_page_count":4},7971461740909,"https://ap-avatar.wpscdn.com/davatar_155a257f0dc6eb9ab79c44ca47cae57d","REVIEW SERIES  \nVon Willebrand disease: classification and epidemiology  \nGiancarlo Castaman1 and Augusto Bramante Federici2  \n1Center for Bleeding Disorders and Coagulation, Department of Heart, Lung and Vessels, Careggi University Hospital, Florence and 2 Hematology and Transfusion Medicine, Luigi Sacco University Hospital, School of Medicine, University of Milan, Milan, Italy  \nCorrespondence: G. Castaman  \n[castaman@aou-careggi.toscana.it](castaman@aou-careggi.toscana.it)  \n[Received:](Received: April 30)[ April 30](Received: April 30), 2025.  \nAccepted: June 25, 2025.  \n[https://doi.org/10.3324/haematol.2024.286058](https://doi.org/10.3324/haematol.2024.286058)  \n©2026 Ferrata Storti Foundation  \nPublished under a CC BY-NC license   \nAbstract  \nVon Willebrand disease (VWD) is a highly heterogeneous inherited bleeding disorder caused by reduced levels or activity of von Willebrand factor (VWF) . VWD is associated with a wide spectrum of clinical and laboratory phenotypes, but diagnosis is based on three main criteria: a positive bleeding history, low levels of circulating VWF and autosomal inheritance patterns. VWD is classified into quantitative VWF deficiencies (type 1, partial and type 3, almost total) and peculiar qualitative defects (type 2 A, B, M, and N) . The prevalence of VWD has been estimated by epidemiological investigations to be ~1%, but a clearcut diagnosis in clinical practice is often not possible because of several confounding factors influencing plasma VWF levels, especially in mild cases. The prevalence of clinically relevant VWD could range from 1/1,000-10,000 inhabitants based on patients referred to tertiary care centers. Recent genetic prevalence data however suggest that pathogenic VWF variants or putative disease alleles may be significantly more common than 1% .  \nIntroduction  \nIn 1926 Erik von Willebrand investigated a family with anew bleeding disorder, named thereafter von Willebrand disease (VWD) in recognition of his pioneering discovery.1 At that time, he was unaware that he was studying what has been recognized 100 years later as the most frequent inherited hemorrhagic disorder. VWD should always be considered first in the differential diagnosis of patients presenting with mild bleeding symptoms since the prevalence of this disease was estimated to be around 1% ina population-based epidemiological investigation conducted in Italy in 1987.2 However, a clear-cut diagnosis of VWD often remains difficult or elusive, because of the wide spectrum of clinical and laboratory manifestations as well as variable penetrance and expressivity. In this regard, genetic testing has limited diagnostic value in mild type 1 VWD (in which von Willebrand factor [VWF] levels are >30 U/dL), whereas it remains useful in other types of VWD.  \nFor these reasons, the actual prevalence of clinically significant cases of VWD is uncertain despite the increasing number of diagnostic laboratory tools. Prevalence estimates are in fact critically influenced by the clinical criteria used to select candidate subjects, the populations  \ninvestigated and by the laboratory criteria to confirm the diagnosis. Moreover, the assessment of the bleeding history is often more difficult in an epidemiological context than in a clinical setting. Physicians must rely on a patient’s bleeding history, which is confounded by personal recollection (recall bias), unless the subject has suffered from severe bleeding episodes, possibly leading to hospitalization. Until recently, no clinical tools were available to objectively quantitate mild or intermediate bleeding symptoms, apart from surgical bleeding and menorrhagia.3 The number and severity of symptoms reported by a patient may be influenced by his or her education, social and cultural context, family setting (e.g. , some symptoms may be under-reported by subjects belonging to a bleeding family) and personality, but also by the typeof data ascertainment.4,5 Hence, stringent clinical criteri","cbCaimFtjcWllO65","https://ap.wps.com/l/cbCaimFtjcWllO65","pdf",255789,"English","# Abstract\n# Introduction\n# Classification of von Willebrand disease\n## Quantitative and qualitative VWF abnormalities\n## Type 1 VWD\n## Type 3 VWD\n## Type 2 VWD (qualitative defects)","[{\"question\":\"What criteria are used to diagnose Von Willebrand disease (VWD)?\",\"answer\":\"VWD diagnosis is based on three main criteria: a positive bleeding history, low levels of circulating VWF, and autosomal inheritance patterns.\"},{\"question\":\"How is VWD classified by VWF abnormalities?\",\"answer\":\"VWD is classified into quantitative VWF deficiencies (type 1 and type 3) and qualitative VWF defects (type 2 subtypes A, B, M, and N).\"},{\"question\":\"Why is the prevalence of clinically significant VWD difficult to determine?\",\"answer\":\"Prevalence estimates vary because they depend on clinical criteria used to select subjects, population studied, laboratory criteria for confirmation, and the challenges of assessing bleeding history in epidemiological settings.\"}]","Von Willebrand disease: classification and epidemiology | PDF",1790726022,23]