{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,6,18]],"date-time":"2025-06-18T00:01:02Z","timestamp":1750204862795,"version":"3.37.3"},"reference-count":30,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2018,2,10]],"date-time":"2018-02-10T00:00:00Z","timestamp":1518220800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2018,2,10]],"date-time":"2018-02-10T00:00:00Z","timestamp":1518220800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"},{"start":{"date-parts":[[2018,2,10]],"date-time":"2018-02-10T00:00:00Z","timestamp":1518220800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Helsinki University Hospital competitive research fund"}],"content-domain":{"domain":["wiley.com"],"crossmark-restriction":true},"short-container-title":["Int J Lab Hematology"],"published-print":{"date-parts":[[2018,6]]},"abstract":"Abstract<\/jats:title>Introduction<\/jats:title>Diagnosis of von Willebrand disease (VWD<\/jats:styled-content>) is challenging, particularly for type 1. The current diagnostic guidelines emphasize simultaneous bleeding symptoms and von Willebrand factor (VWF<\/jats:styled-content>) levels of <30\u201040 IU<\/jats:styled-content>\/dL<\/jats:styled-content>. Historical diagnoses require updated evaluation. We assessed the accuracy of past VWD<\/jats:styled-content> diagnoses in our comprehensive care center with the standardized bleeding score (BS<\/jats:styled-content>) and central laboratory analysis, focusing on VWF<\/jats:styled-content>\u2010dependent platelet functions in whole blood.<\/jats:p><\/jats:sec>Methods<\/jats:title>Our study comprised 83 adults with prior VWD<\/jats:styled-content> who were diagnosed a median of 20\u00a0years ago. We assessed BS<\/jats:styled-content>, VWF<\/jats:styled-content> antigen and activity (minimum of 3 measurements), FVIII<\/jats:styled-content>, PFA<\/jats:styled-content>\u2010100\u00ae<\/jats:sup>, and platelet aggregation via Multiplate\u00ae<\/jats:sup>. Genetic testing was targeted to types 3, 2N, 2B, and equivocal cases.<\/jats:p><\/jats:sec>Results<\/jats:title>All 13\/13 (100%) type 3 and 29\/32 (90%) type 2, but only 10\/38 (26%) of type 1 (overall 52\/83 (63%)) patients met the current criteria for VWD<\/jats:styled-content>. All confirmed cases had abnormal BS<\/jats:styled-content>, impaired PFA<\/jats:styled-content>\u2010100\u00ae<\/jats:sup>, and decreased or absent ristocetin\u2010induced platelet aggregation (RIPA<\/jats:styled-content>), except subtype 2B. VWF<\/jats:styled-content>, FVIII<\/jats:styled-content>, RIPA<\/jats:styled-content>, and PFA<\/jats:styled-content> correlated with BS<\/jats:styled-content> including all study subjects. Ten of the 38 patients with previous type 1 had low VWF<\/jats:styled-content> (35\u201050 IU<\/jats:styled-content>\/dL<\/jats:styled-content>) and variable VWF<\/jats:styled-content>\u2010dependent platelet function. Altogether, 21\/83 patients (25%) had repeatedly normal VWF<\/jats:styled-content>:RC<\/jats:styled-content>o (>50 IU<\/jats:styled-content>\/dL<\/jats:styled-content>).<\/jats:p><\/jats:sec>Conclusion<\/jats:title>von Willebrand disease is associated with impaired VWF<\/jats:styled-content>\u2010dependent whole blood platelet functions that match traditional VWF<\/jats:styled-content> measurements. We detected normal VWF<\/jats:styled-content> in 25% of historically diagnosed patients, mainly type 1 patients, implying that there is a need to systematically re\u2010evaluate historical VWD<\/jats:styled-content> diagnoses.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ijlh.12785","type":"journal-article","created":{"date-parts":[[2018,2,10]],"date-time":"2018-02-10T07:32:43Z","timestamp":1518247963000},"page":"304-311","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Comprehensive re\u2010evaluation of historical von Willebrand disease diagnosis in association with whole blood platelet aggregation and function"],"prefix":"10.1111","volume":"40","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5134-7288","authenticated-orcid":false,"given":"V.","family":"Nummi","sequence":"first","affiliation":[{"name":"Faculty of Medicine University of Helsinki Helsinki Finland"},{"name":"Coagulation Disorders Unit Department of Hematology and Comprehensive Cancer Helsinki University Hospital Helsinki Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R.","family":"Lassila","sequence":"additional","affiliation":[{"name":"Faculty of Medicine University of Helsinki Helsinki Finland"},{"name":"Coagulation Disorders Unit Department of Hematology and Comprehensive Cancer Helsinki University Hospital Helsinki Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L.","family":"Joutsi\u2010Korhonen","sequence":"additional","affiliation":[{"name":"Coagulation Disorders Unit Department of Clinical Chemistry HUSLAB Laboratory Services Helsinki University Hospital Helsinki Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E.","family":"Armstrong","sequence":"additional","affiliation":[{"name":"Coagulation Disorders Unit Department of Hematology and Comprehensive Cancer Helsinki University Hospital Helsinki Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"T.","family":"Szanto","sequence":"additional","affiliation":[{"name":"Coagulation Disorders Unit Department of Clinical Chemistry HUSLAB Laboratory Services Helsinki University Hospital Helsinki Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2018,2,10]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2006.02146.x"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1538-7836.2003.00260.x"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2004.00991.x"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0031-1281034"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.13064"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2007.01643.x"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2012.077263"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2015-10-673681"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-2006-947859"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/MBC.0b013e32834a7e01"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0034-1383546"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.thromres.2014.05.020"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0031-1300952"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12710"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12653"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1160\/TH06-05-0242"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2011.04400.x"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1160\/TH17-02-0129"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2006.01847.x"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2516.1999.0050s2072.x"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.thromres.2007.03.022"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1160\/TH07-07-0478"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12225"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2002-09-2892"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2004.00933.x"},{"key":"e_1_2_7_27_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2010.08333.x"},{"key":"e_1_2_7_28_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2004.00693.x"},{"key":"e_1_2_7_29_1","doi-asserted-by":"publisher","DOI":"10.1038\/194927b0"},{"key":"e_1_2_7_30_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12664"},{"key":"e_1_2_7_31_1","doi-asserted-by":"publisher","DOI":"10.1309\/E4947DG48TVY19C2"}],"container-title":["International 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