{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T06:12:04Z","timestamp":1785564724976,"version":"3.56.0"},"reference-count":34,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2022,9,28]],"date-time":"2022-09-28T00:00:00Z","timestamp":1664323200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["European J of Haematology"],"published-print":{"date-parts":[[2022,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>von Willebrand factor ristocetin cofactor (vWF activity) and platelet count (PLT) are negatively correlated in patients with polycythemia vera (PV) and essential thrombocythemia (ET). However, vWF activity does not always normalize upon controlling PLT in those patients. To address this issue, we investigated the correlation between vWF activity and PLT in PV and ET patients. The negative correlation between vWF activity and PLT was stronger in <jats:italic>calreticulin<\/jats:italic> mutation\u2010positive (<jats:italic>CALR<\/jats:italic>+) ET than in <jats:italic>Janus kinase 2<\/jats:italic> mutation\u2010positive (<jats:italic>JAK2<\/jats:italic>+) PV or ET groups. When PLT were maintained at a certain level (&lt;600\u2009\u00d7\u200910<jats:sup>9<\/jats:sup>\/L), low vWF activity (&lt;50%) was more frequently observed in <jats:italic>JAK2<\/jats:italic>+ PV patients than in <jats:italic>JAK2<\/jats:italic>+ ET (<jats:italic>p<\/jats:italic>\u00a0=\u00a0.013) or <jats:italic>CALR<\/jats:italic>+ ET (<jats:italic>p<\/jats:italic>\u00a0=\u00a0.013) groups, and in PV and ET patients with \u226550% <jats:italic>JAK2<\/jats:italic>+ allele burden than in those with allele burden &lt;50% (<jats:italic>p<\/jats:italic>\u00a0=\u00a0.015). High vWF activity (&gt;150%) was more frequent in the <jats:italic>JAK2<\/jats:italic>+ ET group than in the <jats:italic>CALR<\/jats:italic>+ ET group (<jats:italic>p<\/jats:italic>\u00a0=\u00a0.005), and often associated with vasomotor symptoms (<jats:italic>p<\/jats:italic>\u00a0=\u00a0.002). This study suggests that some patients with <jats:italic>JAK2<\/jats:italic>+ PV or ET have vWF activity outside the standard range even with well\u2010controlled PLT, and that the measurement of vWF activity is useful for assessing the risk of thrombosis and hemorrhage.<\/jats:p>","DOI":"10.1111\/ejh.13866","type":"journal-article","created":{"date-parts":[[2022,9,22]],"date-time":"2022-09-22T02:43:40Z","timestamp":1663814620000},"page":"779-786","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["von Willebrand factor activity levels are influenced by driver mutation status in polycythemia vera and essential thrombocythemia patients with well\u2010controlled platelet counts"],"prefix":"10.1111","volume":"109","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4979-3000","authenticated-orcid":false,"given":"Kazuhide","family":"Iizuka","sequence":"first","affiliation":[{"name":"Division of Laboratory Medicine, 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