{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T18:56:28Z","timestamp":1791399388954,"version":"4.3.3"},"reference-count":22,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2020,12,30]],"date-time":"2020-12-30T00:00:00Z","timestamp":1609286400000},"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":[[2021,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objectives<\/jats:title><jats:p>In patients with essential thrombocythemia (ET), after the <jats:italic>JAK2<\/jats:italic>V617F driver mutation, mutations in <jats:italic>CALR<\/jats:italic> are common (classified as type 1, 52\u2010bp deletion or type 2, 5\u2010bp insertion). <jats:italic>CALR<\/jats:italic> mutations have generally been associated with a lower risk of thrombosis. This study aimed to confirm the impact of <jats:italic>CALR<\/jats:italic> mutation type on thrombotic risk.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We retrospectively investigated 983 ET patients diagnosed in Spanish and Polish hospitals.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>With 7.5\u00a0years of median follow\u2010up from diagnosis, 155 patients (15.8%) had one or more thrombotic event. The 5\u2010year thrombosis\u2010free survival (TFS) rate was 83.8%, 91.6% and 93.9% for the <jats:italic>JAK2<\/jats:italic>V617F, <jats:italic>CALR<\/jats:italic>\u2010type 1 and <jats:italic>CALR<\/jats:italic>\u2010type 2 groups, respectively (<jats:italic>P<\/jats:italic>\u00a0=\u00a0.002). Comparing <jats:italic>CALR<\/jats:italic>\u2010type 1 and <jats:italic>CALR<\/jats:italic>\u2010type 2 groups, TFS for venous thrombosis was lower in <jats:italic>CALR<\/jats:italic>\u2010type 1 (<jats:italic>P<\/jats:italic>\u00a0=\u00a0.046), with no difference in TFS for arterial thrombosis observed. The cumulative incidence of thrombosis was significantly different comparing <jats:italic>JAK2<\/jats:italic>V617F vs <jats:italic>CALR<\/jats:italic>\u2010type 2 groups but not <jats:italic>JAK2<\/jats:italic>V617F vs <jats:italic>CALR<\/jats:italic>\u2010type 1 groups. Moreover, <jats:italic>CALR\u2010<\/jats:italic>type 2 mutation was a statistically significant protective factor for thrombosis with respect to <jats:italic>JAK2<\/jats:italic>V617F in multivariate logistic regression (OR: 0.45, <jats:italic>P<\/jats:italic>\u00a0=\u00a0.04) adjusted by age.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Our results suggest that <jats:italic>CALR<\/jats:italic> mutation type has prognostic value for the stratification of thrombotic risk in ET patients.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ejh.13561","type":"journal-article","created":{"date-parts":[[2020,12,4]],"date-time":"2020-12-04T19:55:20Z","timestamp":1607111720000},"page":"371-379","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["The risk of thrombosis in essential thrombocythemia is associated with the type of <i>CALR<\/i> mutation: A multicentre collaborative 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