{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T02:28:50Z","timestamp":1791340130880,"version":"4.3.1"},"reference-count":24,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2016,4,4]],"date-time":"2016-04-04T00:00:00Z","timestamp":1459728000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"The Mayo Clinic Harvey-Yulman Charitable Foundation for Myelofibrosis Tissue Bank, Mayo Clinic, Rochester, MN, USA"},{"name":"Clinical Database of Molecular and Biological Abnormalities, Mayo Clinic, Rochester, MN, USA"},{"name":"The Henry J. Predolin Foundation for Research in Leukemia, Mayo Clinic, Rochester, MN, USA"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2016,5]]},"abstract":"<jats:p>About 85% of patients with essential thrombocythemia (ET) harbor one of three driver mutations: <jats:italic>JAK2<\/jats:italic>, calreticulin (<jats:italic>CALR<\/jats:italic>), and <jats:italic>MPL<\/jats:italic>; the remaining ( \u223c15%) are wild type for all three mutations and are referred to as being \u201ctriple negative.\u201d Furthermore, <jats:italic>CALR<\/jats:italic> mutations in ET are structurally classified as type 1\/type 1\u2010like or type 2\/type 2\u2010like variants. The objective of the current study was to examine the impact of <jats:italic>CALR<\/jats:italic> mutation variant stratified driver mutational status on overall (OS), myelofibrosis\u2010free (MFFS), thrombosis\u2010free, and leukemia\u2010free survival (LFS) in ET; 495 patients (median age 58 years; 61% females) with ET were fully annotated for the their driver mutational status: 321 (65%) harbored <jats:italic>JAK2<\/jats:italic>, 109 (22%) <jats:italic>CALR<\/jats:italic>, and 12 (2%) <jats:italic>MPL<\/jats:italic> mutations and 11% were triple\u2010negative. Among the 109 <jats:italic>CALR<\/jats:italic>\u2010mutated cases, 52% were classified as type 1\/type 1\u2010like and 48% as type 2\/type 2\u2010like. In univariate analysis, triple\u2010negative patients displayed the best and <jats:italic>MPL<\/jats:italic> mutated the worst OS (<jats:italic>P<\/jats:italic>\u2009=\u20090.007); however, the difference in OS was no longer apparent on multivariable analysis that included age and sex as covariates (<jats:italic>P<\/jats:italic>\u2009=\u20090.5). LFS was also similar among the different mutational groups (<jats:italic>P<\/jats:italic>\u2009=\u20090.6) whereas MFFS was significantly shorter in <jats:italic>MPL<\/jats:italic>\u2010mutated patients on both univariate and multivariable analyses (age\u2010adjusted <jats:italic>P<\/jats:italic>\u2009=\u20090.02; HR 7.9, 95% CI 2.0\u201331.5). Also in multivariable analysis that included thrombosis history, age, and cardiovascular risk factors, the presence of <jats:italic>JAK2<\/jats:italic> or <jats:italic>MPL<\/jats:italic> mutations was independently associated with higher risk of thrombosis (<jats:italic>P<\/jats:italic>\u2009=\u20090.02; HR 1.9, 95% CI 1.1\u20133.4). In conclusion, driver mutational status in ET does not appear to influence overall or LFS, even after <jats:italic>CALR<\/jats:italic> variant stratification. However, the presence of <jats:italic>MPL<\/jats:italic> mutations might be associated with a higher risk of fibrotic transformation and the presence of <jats:italic>JAK2<\/jats:italic>\/<jats:italic>MPL<\/jats:italic> mutations with higher risk of thrombosis. Am. J. Hematol. 91:503\u2013506, 2016. \u00a9 2016 Wiley Periodicals, Inc.<\/jats:p>","DOI":"10.1002\/ajh.24338","type":"journal-article","created":{"date-parts":[[2016,2,19]],"date-time":"2016-02-19T00:18:31Z","timestamp":1455841111000},"page":"503-506","source":"Crossref","is-referenced-by-count":54,"title":["Calreticulin variant stratified driver mutational status and prognosis in essential thrombocythemia"],"prefix":"10.1002","volume":"91","author":[{"given":"Yoseph C.","family":"Elala","sequence":"first","affiliation":[{"name":"Division of Hematology Mayo Clinic Rochester Minnesota"},{"name":"Department of Medicine Mayo Clinic Rochester Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Terra L.","family":"Lasho","sequence":"additional","affiliation":[{"name":"Division of Hematology Mayo Clinic Rochester Minnesota"},{"name":"Department of Medicine Mayo Clinic Rochester 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Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Animesh","family":"Pardanani","sequence":"additional","affiliation":[{"name":"Division of Hematology Mayo Clinic Rochester Minnesota"},{"name":"Department of Medicine Mayo Clinic Rochester Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ayalew","family":"Tefferi","sequence":"additional","affiliation":[{"name":"Division of Hematology Mayo Clinic Rochester Minnesota"},{"name":"Department of Medicine Mayo Clinic Rochester Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2016,4,4]]},"reference":[{"key":"e_1_2_5_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/leu.2014.35"},{"key":"e_1_2_5_3_1","first-page":"937","author":"Vardiman JW","year":"2009","journal-title":"The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important 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