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The series connects the historical “natural experiment” with modern understanding of von Willebrand factor’s quantitative and qualitative defects, its roles in primary and secondary hemostasis, and advances in clinical, laboratory, and molecular characterization. It highlights selected landmark contributions and outlines topics including classification, genetic testing, treatments, and women’s gynecologic and obstetric management.",{"@graph":69,"@context":122},[70,84,105],{"@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/introduction-to-a-review-series-a-century-after-erik-von-willebrand/448276/",{"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/introduction-to-a-review-series-a-century-after-erik-von-willebrand/448276.png","ImageObject",300,407,{"name":92,"@type":93},"River Wang","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-10-30","2026-09-30",true,{"@type":102,"interactionType":103,"userInteractionCount":44},"InteractionCounter",{"@type":104},"ViewAction",{"@type":106,"mainEntity":107},"FAQPage",[108,114,118],{"name":109,"@type":110,"acceptedAnswer":111},"What is the main focus of this review series introduction?","Question",{"text":112,"@type":113},"It reviews a century of progress since Erik von Willebrand’s first description of von Willebrand disease and frames the series’ subsequent articles and themes.","Answer",{"name":115,"@type":110,"acceptedAnswer":116},"What causes von Willebrand disease according to the introduction?",{"text":117,"@type":113},"It results from quantitative and/or qualitative defects of von Willebrand factor (VWF), a multimeric glycoprotein involved in primary and secondary hemostasis.",{"name":119,"@type":110,"acceptedAnswer":120},"Which areas do the listed review articles cover?",{"text":121,"@type":113},"They cover landmark historical contributions, VWF structure and functions, clinical and laboratory diagnosis, classification and epidemiology, molecular genetic testing, treatment history and future options, and gynecologic/obstetric management for girls and women.","https://schema.org",{"og:url":83,"og:type":124,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":126,"canonical":83},"index,follow",{"doc_id":128,"site_id":62},448276,1790817007,{"code":4,"msg":5,"data":131},{"doc_id":128,"user_id":132,"nickname":92,"user_avatar":133,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":134,"file_id":135,"file_url":136,"file_type":137,"file_size":138,"view_count":44,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":24,"language":139,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":140,"faqs":141,"seo_title":142,"seo_description":67,"update_tm":143,"read_time":144,"preview_page_count":4},1099514067438,"https://ap-avatar.wpscdn.com/avatar/100002539ee87300030?x-image-process=image/resize,m_fixed,w_180,h_180&k=1780474512215547542","INTRODUCTION TO A REVIEW SERIES  \nIntroduction to the Review Series. A century after Erik von Willebrand  \nPier Mannuccio Mannucci1 and Francesco Rodeghiero2  \n1 Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan and 2 Hematology Project Foundation, affiliated to the Department of Hematology, San Bortolo Hospital, Vicenza Italy  \nCorrespondence: P. M. Mannucci  \n[piermannuccio.mannucci@policlinico.mi.it](piermannuccio.mannucci@policlinico.mi.it)  \n[Received:](Received: June 27)[ June 27](Received: June 27), 2025.  \nAccepted: July 3, 2025.  \n[https://doi.org/10.3324/haematol.2024.286060](https://doi.org/10.3324/haematol.2024.286060)  \n©2026 Ferrata Storti Foundation  \nPublished under a CC BY-NC license   \nAs in many other cases in the history of medical advances, a natural experiment was at the origin of the first description of von Willebrand disease (VWD), the most frequent inherited bleeding disorder. VWD is caused by quantitative and/or qualitative defects of von Willebrand factor (VWF), a huge multimeric glycoprotein produced by endothelial cells and megakaryocytes and endowed with multiple functions in primary and secondary hemostasis. The first case was published in 1926 in Swedish language by Erik von Willebrand, professor of medicine at Helsinki University in Finland. In the century since his first description of anew disease (that he called hereditary pseudohemophilia and distinguished from hemophilia), a large number of publications fostered the understanding of this complex and common bleeding disorder. The clinical and molecular heterogeneity of VWD and knowledge on the multiple biological functions of VWF witnessed dramatic advances, which are featured in the frame of a review series by means of the following articles and authors:  \n• Riitta Lassila and Erik Berntorp,“The landmark contribution by Erik von Willebrand”;1  \n• Sandra Haberichter and S. James O’Donnell,“Structure and multiple functions of von Willebrand factor”;2  \n• Alberto Tosetto and Jeroen Eikenboom,“Clinical and laboratory diagnosis of von Willebrand disease”;3  \n• Giancarlo Castaman and Augusto Federici,“Von Willebrand disease: classification and epidemiology”;4  \n• Omid Seidizadeh and David Lillicrap,“Molecular genetic testing in von Willebrand disease: past, present and beyond”;5  \n• Caterina Casari, Frank Leebeek and Flora Peyvandi,“Historical, recent and future treatments for von Willebrand disease”;6  \n• Rezan Abdul-Kadir and Margaret Ragni,“Gynecologic and obstetric management of girls and women with von Willebrand disease”.7  \nFor the first article of the review series we chose Erik Berntorp from Malmo and Riitta Lassila from Helsinki in order  \nto emphasize and celebrate at the same time Finland, asthe country of the author of the first description of VWD, as well as Sweden, where the pioneering work of Inge Maria Nilsson and Margareta Blombäck contributed enormously to knowledge about the disease and early treatment. The article reiterates the features of the index case, i.e., Hjordis Sundblom, a young woman from Föglö in the Åland island archipelago. Incidentally, a few years ago we both visited the cemetery of Föglö, which contains Hjordis’ grave, as well asthe house where she lived with her large family including at least seven additional bleeders, some of whom died prematurely of catastrophic hemorrhages.8 Hjordis suffered from type 3 VWD, a form compatible with such a severe clinical phenotype that she died from exsanguination at the age of 14 years at the time of her fourth menstrual period. Hjordis was referred to Erik von Willebrand’s clinic and had to go to Deaconess Hospital in Helsinki repeatedly during as many as 9 years because of severe bleeding episodes in multiple sites and mucosal tracts. Her frequent pilgrimages by boat from Föglö to Helsinki bear witness to the limited therapeutic facilities available at the time, practically only vitamins and whole ","cbCaiutL2X21DCWt","https://ap.wps.com/l/cbCaiutL2X21DCWt","pdf",118602,"English","# Introduction to a Review Series\n## Historical origins of von Willebrand disease\n## Von Willebrand factor functions in hemostasis\n## Selected landmark articles in the review series\n## Clinical case highlights and laboratory diagnosis\n## Molecular advances and future directions","[{\"question\":\"What is the main focus of this review series introduction?\",\"answer\":\"It reviews a century of progress since Erik von Willebrand’s first description of von Willebrand disease and frames the series’ subsequent articles and themes.\"},{\"question\":\"What causes von Willebrand disease according to the introduction?\",\"answer\":\"It results from quantitative and/or qualitative defects of von Willebrand factor (VWF), a multimeric glycoprotein involved in primary and secondary hemostasis.\"},{\"question\":\"Which areas do the listed review articles cover?\",\"answer\":\"They cover landmark historical contributions, VWF structure and functions, clinical and laboratory diagnosis, classification and epidemiology, molecular genetic testing, treatment history and future options, and gynecologic/obstetric management for girls and women.\"}]","Introduction to a Review Series - A century after Erik von Willebrand | PDF",1790726406,13]