{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,7]],"date-time":"2026-11-07T10:32:04Z","timestamp":1794047524592,"version":"4.8.0"},"reference-count":39,"publisher":"Ferrata Storti Foundation (Haematologica)","issue":"1","license":[{"start":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T00:00:00Z","timestamp":1767225600000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["haematol"],"abstract":"<jats:p>One hundred years ago Professor Erik von Willebrand, working at the Deaconess Hospital in Helsinki, Finland, published his landmark paper on hereditary pseudohemophilia (the original paper, in Swedish, was entitled Heredit\u00e4r pseudohemofili and was published in Finska L\u00e4kares\u00e4llskapets Handlingar). In 1924, a 5-year-old girl named Hj\u00f6rdis, living in F\u00f6gl\u00f6 in the \u00c5land Island archipelago, was brought to Erik von Willebrand\u2019s attention; his investigations of a large family living in the \u00c5land Islands emerged in the 1926 publication in which a new hereditary bleeding disorder, distinct from classic hemophilia, was described. The index case was the ninth of 11 siblings of whom three had already bled to death. Hj\u00f6rdis herself had a history of serious bleeding, including nose bleeds, hematomas, anemia and, also, an ankle bleed. At the age of 14 years, she drastically bled to death during her fourth menstruation. Erik von Willebrand came close with his interpretation of the disorder, but it took several decades until other investigators could fully elucidate the pathophysiology underlying the bleeding diathesis which nowadays is known as von Willebrand disease. The index case had the most severe subtype 3. The structure, function and genetics of the factor named von Willebrand factor have now been revealed and the symptoms, epidemiology and treatment of the disorder thoroughly studied. The pioneering and visionary work of Erik von Willebrand, combining laboratory methods and clinical sharpness, set the stage for improving the lives of numerous people suffering from a generalized bleeding tendency or even life-threatening bleeds due to this most common hereditary bleeding disorder.<\/jats:p>","DOI":"10.3324\/haematol.2024.286012","type":"journal-article","created":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T12:16:17Z","timestamp":1767269777000},"page":"1-9","source":"Crossref","is-referenced-by-count":4,"title":["The landmark contribution by Erik von Willebrand"],"prefix":"10.3324","volume":"111","author":[{"given":"Riitta","family":"Lassila","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erik","family":"Berntorp","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1856","published-online":{"date-parts":[[2026,1,1]]},"reference":[{"key":"82968","unstructured":"von Willebrand EA. 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Blood Rev. 2010; 24(3):123-134.","DOI":"10.1016\/j.blre.2010.03.003"},{"key":"82974","doi-asserted-by":"crossref","unstructured":"Zhang ZP, Blomback M, Egberg N, Falk G, Anvret M. Characterization of the von Willebrand factor gene (VWF) in von Willebrand disease type III patients from 24 families of Swedish and Finnish origin. Genomics. 1994; 21(1):188-193.","DOI":"10.1006\/geno.1994.1241"},{"key":"82975","doi-asserted-by":"crossref","unstructured":"Zhang ZP, Blomback M, Nyman D, Anvret M. Mutations of von Willebrand factor gene in families with von Willebrand disease in the Aland Islands. Proc Natl Acad Sci U S A. 1993; 90(17):7937-7940.","DOI":"10.1073\/pnas.90.17.7937"},{"key":"82976","doi-asserted-by":"crossref","unstructured":"Sanders YV, Giezenaar MA, Laros-van Gorkom BA. von Willebrand disease and aging: an evolving phenotype. J Thromb Haemost. 2014; 12(7):1066-1075.","DOI":"10.1111\/jth.12586"},{"key":"82977","unstructured":"von Willebrand EA, Jurgens R, Dahlberg U. Konstitutionell trombopati, en ny \u00e4rftlig bl\u00f6darsjukdom. Fin L\u00e4kares\u00e4llsk Handl. 1934; LXXVI:193-232."},{"key":"82978","doi-asserted-by":"crossref","unstructured":"Gallinaro L, Cattini MG, Sztukowska M. A shorter von Willebrand factor survival in O blood group subjects explains how ABO determinants influence plasma von Willebrand factor. Blood. 2008; 111(7):3540-3545.","DOI":"10.1182\/blood-2007-11-122945"},{"key":"82979","doi-asserted-by":"crossref","unstructured":"Fischer K, Pendu R, van Schooten CJ. Models for prediction of factor VIII half-life in severe haemophiliacs: distinct approaches for blood group O and non-O patients. PLoS One. 2009; 4(8):e6745.","DOI":"10.1371\/journal.pone.0006745"},{"key":"82980","unstructured":"von Willebrand E, Jurgens R. Uber eine neues vererbbares Blutungsubel: die konstitutionelleThrombopathie. 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