[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"detail-sidebar-cat-0-en-105":3,"doc-seo-448220-105":59,"doc-detail-448220-en":131},{"code":4,"msg":5,"data":6},0,"success",[7,13,18,23,28,33,38,43,48,51,55],{"id":8,"doc_module":4,"doc_module_name":9,"category_name":10,"show_sort_weight":11,"slug":12},1,"Document","Story & Novel",90,"story-novel",{"id":14,"doc_module":4,"doc_module_name":9,"category_name":15,"show_sort_weight":16,"slug":17},2,"Literature",80,"literature",{"id":19,"doc_module":4,"doc_module_name":9,"category_name":20,"show_sort_weight":21,"slug":22},4,"Exam",70,"exam",{"id":24,"doc_module":4,"doc_module_name":9,"category_name":25,"show_sort_weight":26,"slug":27},5,"Comic",60,"comic",{"id":29,"doc_module":4,"doc_module_name":9,"category_name":30,"show_sort_weight":31,"slug":32},6,"Technology",50,"technology",{"id":34,"doc_module":4,"doc_module_name":9,"category_name":35,"show_sort_weight":36,"slug":37},7,"Healthcare",40,"healthcare",{"id":39,"doc_module":4,"doc_module_name":9,"category_name":40,"show_sort_weight":41,"slug":42},8,"Research & Report",30,"research-report",{"id":44,"doc_module":4,"doc_module_name":9,"category_name":45,"show_sort_weight":46,"slug":47},9,"Religion & Spirituality",20,"religion-spirituality",{"id":46,"doc_module":4,"doc_module_name":9,"category_name":49,"show_sort_weight":46,"slug":50},"World Cup","world-cup",{"id":52,"doc_module":4,"doc_module_name":9,"category_name":53,"show_sort_weight":52,"slug":54},10,"Lifestyle","lifestyle",{"id":56,"doc_module":4,"doc_module_name":9,"category_name":57,"show_sort_weight":24,"slug":58},19,"General","general",{"code":4,"msg":60,"data":61},"ok",{"site_id":62,"language":63,"slug":64,"title":65,"keywords":66,"description":67,"schema_data":68,"social_meta":124,"head_meta":126,"extra_data":128,"updated_unix":130},105,"en","clinical-and-laboratory-diagnosis-of-von-willebrand-disease-review-series","Clinical and laboratory diagnosis of von Willebrand disease - Review Series","","von Willebrand disease (VWD) is a hereditary bleeding disorder first described in 1926 and marked by frequent bruising, mucocutaneous and procedural bleeding, gastrointestinal bleeding, heavy menstrual bleeding, and joint bleeds. The disorder results from deficiency or dysfunction of von Willebrand factor (VWF), a high-molecular-weight protein that circulates with factor VIII. Diagnosis relies on a clinical bleeding history, abnormal VWF function testing, and demonstration of familial inheritance, yet the variable phenotype makes evaluation complex. A Bayesian integration of clinical and laboratory data can support diagnosis, while laboratory testing progresses from screening and first-level assays to second-level subtyping for type 2 variants, enabling proper classification and treatment planning while reducing overdiagnosis.",{"@graph":69,"@context":123},[70,84,106],{"@type":71,"itemListElement":72},"BreadcrumbList",[73,77,79,82],{"item":74,"name":75,"@type":76,"position":8},"https://docshare.wps.com","Home","ListItem",{"item":78,"name":9,"@type":76,"position":14},"https://docshare.wps.com/document/",{"item":80,"name":35,"@type":76,"position":81},"https://docshare.wps.com/document/healthcare/",3,{"item":83,"name":65,"@type":76,"position":19},"https://docshare.wps.com/document/clinical-and-laboratory-diagnosis-of-von-willebrand-disease-review-series/448220/",{"url":83,"name":65,"@type":85,"image":86,"author":91,"headline":65,"publisher":94,"fileFormat":97,"inLanguage":63,"description":67,"dateModified":98,"datePublished":99,"encodingFormat":97,"isAccessibleForFree":100,"interactionStatistic":101},"DigitalDocument",{"url":87,"@type":88,"width":89,"height":90},"https://docshare.wps.com/thumbnails/clinical-and-laboratory-diagnosis-of-von-willebrand-disease-review-series/448220.png","ImageObject",300,407,{"name":92,"@type":93},"Levi","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-10-30","2026-09-29",true,{"@type":102,"interactionType":103,"userInteractionCount":105},"InteractionCounter",{"@type":104},"ViewAction",13,{"@type":107,"mainEntity":108},"FAQPage",[109,115,119],{"name":110,"@type":111,"acceptedAnswer":112},"What clinical features define von Willebrand disease in practice?","Question",{"text":113,"@type":114},"Diagnosis considers a bleeding history with typical symptoms, such as bruising, nosebleeds, heavy menstrual bleeding, bleeding after tooth extraction, gastrointestinal bleeding, and joint bleeds. Familial inheritance further supports the clinical picture.","Answer",{"name":116,"@type":111,"acceptedAnswer":117},"How do laboratory tests contribute to diagnosing VWD?",{"text":118,"@type":114},"Laboratory diagnosis requires at least one abnormality of VWF function in tests. Screening and first-level assays measure VWF antigen, VWF-platelet binding activity, and factor VIII coagulant activity, followed by second-level subtyping for type 2 variants.",{"name":120,"@type":111,"acceptedAnswer":121},"Why can the clinical phenotype and laboratory phenotype differ in VWD?",{"text":122,"@type":114},"The relationship between bleeding symptoms and reduced VWF levels may become evident only at much lower VWF ranges. Variable penetrance and age-related improvement in low VWF help explain dissociation between clinical and laboratory findings.","https://schema.org",{"og:url":83,"og:type":125,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":127,"canonical":83},"index,follow",{"doc_id":129,"site_id":62},448220,1790765888,{"code":4,"msg":5,"data":132},{"doc_id":129,"user_id":133,"nickname":92,"user_avatar":134,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":135,"file_id":136,"file_url":137,"file_type":138,"file_size":139,"view_count":105,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":44,"language":140,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":141,"faqs":142,"seo_title":143,"seo_description":67,"update_tm":144,"read_time":145,"preview_page_count":4},7971461740909,"https://ap-avatar.wpscdn.com/davatar_155a257f0dc6eb9ab79c44ca47cae57d","REVIEW SERIES  \nClinical and laboratory diagnosis of von Willebrand disease  \nAlberto Tosetto1 and Jeroen Eikenboom2  \n1 Hemophilia and Thrombosis Center, Hematology Department, San Bortolo Hospital, Vicenza, Italy and 2 Department of Internal Medicine, Division of Thrombosis and Hemostasis, Leiden University Medical Center, Leiden, the Netherlands  \nCorrespondence: A. Tosetto, [alberto.tosetto@aulss8.veneto.it](alberto.tosetto@aulss8.veneto.it)  \n[Received:](Received: April 3)[ April 3](Received: April 3), 2025.  \nAccepted: May 23, 2025.  \n[https://doi.org/10.3324/haematol.2024.286011](https://doi.org/10.3324/haematol.2024.286011)  \n©2026 Ferrata Storti Foundation  \nPublished under a CC BY-NC license   \nAbstract  \nvon Willebrand disease (VWD) is a hereditary bleeding disorder first described by Erik von Willebrand in 1926. The disease is characterized by frequent bruising, bleeding from minor wounds, nosebleeds, heavy menstrual bleeding, bleeding after tooth extraction, gastrointestinal bleeding, and joint bleeds. The underlying cause of VWD was identified 45 years later asa deficiency of von Willebrand factor (VWF), a high-molecular-weight protein that circulates with factor VIII in plasma. The clinical diagnosis of VWD involves the presence of bleeding symptoms, detection of at least one abnormality of VWF function in laboratory tests, and demonstration of familial inheritance. VWD presents a broad spectrum of clinical and laboratory abnormalities, making the diagnostic process complex. Integrating clinical and laboratory data into the diagnostic pathway using a Bayesian approach can help build evidence for a diagnosis. Over the years, several diagnostic assays have been developed to measure the quantitative and qualitative properties of VWF. Automated laboratory assays for measuring VWF have been implemented in recent decades, and assessment tools for clinical evaluation of bleeding severity have been developed. Laboratory diagnosis involves screening and first-level diagnostic tests to measure VWF antigen, VWF-platelet binding activity, and factor VIII coagulant activity. Second-level subtyping tests are required to characterize the phenotypic defects of the type 2 variants and classify the patients. Proper diagnosis and classification of VWD are essential in order to recognize patients who may benefit from treatment, determine the optimal treatment modality, and prevent overdiagnosis.  \nIntroduction  \nWhen Erik von Willebrand described in 1926 a new hemorrhagic syndrome that he had recognized in a family from the town of Föglo, in the Åland Islands, he did not have standardized bleeding questionnaires or bleeding assessment tools at his disposal, nor any of the sophisticated laboratory tests that we can nowadays apply to make the diagnosis of von Willebrand disease (VWD) 1,2 The clinical symptoms encountered in the family described by von Willebrand were frequent bruising, bleeding from minor wounds, nosebleeds, heavy menstrual bleeding, bleeding after tooth extraction, gastrointestinal bleeding, and joint bleeds. The bleeds could be severe and, in several cases, fatal. He recognized the familial nature of the disease but was uncertain about the cause and named it “hereditary pseudo-hemophilia.”1 Forty-five years later, the disease was  \nassociated with a deficiency of a high-molecular-weight protein, von Willebrand factor (VWF), independently synthesized and secreted from factor VIII (FVIII) but tightly bound to it in plasma.3  \nSince the elucidation of the main pathophysiology of the disease, several diagnostic assays have been developed over the years to measure quantitative and qualitative properties of VWF. New automated laboratory assays for measuring VWF have been developed and implemented in recent decades.4 By the early 2000s, the development of assessment tools for clinical evaluation of bleeding severity had been addressed.5,6 Proper diagnosis and classification of VWD are essential to recognize patients who m","cbCaivrxbhziDLIr","https://ap.wps.com/l/cbCaivrxbhziDLIr","pdf",241197,"English","# Abstract\n# Introduction\n# Clinical diagnosis of von Willebrand disease\n# Integrating clinical and laboratory data into the diagnostic pathway","[{\"question\":\"What clinical features define von Willebrand disease in practice?\",\"answer\":\"Diagnosis considers a bleeding history with typical symptoms, such as bruising, nosebleeds, heavy menstrual bleeding, bleeding after tooth extraction, gastrointestinal bleeding, and joint bleeds. Familial inheritance further supports the clinical picture.\"},{\"question\":\"How do laboratory tests contribute to diagnosing VWD?\",\"answer\":\"Laboratory diagnosis requires at least one abnormality of VWF function in tests. Screening and first-level assays measure VWF antigen, VWF-platelet binding activity, and factor VIII coagulant activity, followed by second-level subtyping for type 2 variants.\"},{\"question\":\"Why can the clinical phenotype and laboratory phenotype differ in VWD?\",\"answer\":\"The relationship between bleeding symptoms and reduced VWF levels may become evident only at much lower VWF ranges. Variable penetrance and age-related improvement in low VWF help explain dissociation between clinical and laboratory findings.\"}]","Clinical and laboratory diagnosis of von Willebrand disease - Review Series | PDF",1790726096,23]