{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,31]],"date-time":"2026-08-31T18:31:51Z","timestamp":1788201111528,"version":"build-2803163510"},"reference-count":40,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2025,9,3]],"date-time":"2025-09-03T00:00:00Z","timestamp":1756857600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2025,11]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Survival prediction models in essential thrombocythemia (ET) include the International Prognostic Scoring System (IPSET) and the more recently introduced triple\u2010A (AAA) prognostic score. The latter enlists age and absolute neutrophil (ANC) and lymphocyte (ALC) counts as risk variables. In the current study, a Mayo Clinic discovery cohort of 658 patients with ET was used to identify AAA\u2010independent risk variables. Accordingly, multivariable analysis\u2010derived HRs (95% CI) were 15.7 (8.4\u201329.5) for age\u2009&gt;\u200970\u2009years (8 points); 4.2 (2.3\u20137.5) for age 50 to 70\u2009years (2 points); 1.8 (1.2\u20132.5) for ANC \u2265\u20098\u2009\u00d7\u200910\n                    <jats:sup>9<\/jats:sup>\n                    \/L (1 point); 1.4 (1.03\u20131.9) for ALC &lt;\u20091.7\u2009\u00d7\u200910\n                    <jats:sup>9<\/jats:sup>\n                    \/L (1 point); 1.8 (1.2\u20132.6) for absolute monocyte count (AMC) \u2265\u20090.5\u2009\u00d7\u200910\n                    <jats:sup>9<\/jats:sup>\n                    \/L (1 point); 1.8 (1.2\u20132.3) for male sex (1 point); 1.8 (1.3\u20132.4) for arterial hypertension (1 point); and 1.6 (1.2\u20132.3) for arterial thrombosis (1 point). HR\u2010weighted scoring enabled a 4\u2010tiered risk classification: ultra\u2010low (0\u20131 points;\n                    <jats:italic>N<\/jats:italic>\n                    \u2009=\u200994; median survival 42.7\u2009years), low (2\u20134 points;\n                    <jats:italic>N<\/jats:italic>\n                    \u2009=\u2009297; 23\u2009years), intermediate (5 points;\n                    <jats:italic>N<\/jats:italic>\n                    \u2009=\u200966; 17.3\u2009years), and high (6\u201314 points;\n                    <jats:italic>N<\/jats:italic>\n                    \u2009=\u2009201; 10.8\u2009years). Time\u2010dependent predictive performance at 20\/25\u2009years favored AAA\n                    <jats:sup>+<\/jats:sup>\n                    (AUC 0.92\/0.91) vs. AAA (0.86\/0.86) vs. IPSET (0.81\/0.84). The AAA\n                    <jats:sup>+<\/jats:sup>\n                    risk model was subsequently validated by two external cohorts from Israel (\n                    <jats:italic>N<\/jats:italic>\n                    \u2009=\u20095968) and Italy (\n                    <jats:italic>N<\/jats:italic>\n                    \u2009=\u2009682). In the cohort from Israel, disease\u2010specific mortality was assessed by comparing observed survival to an age\u2010 and sex\u2010matched reference population, which suggested near\u2010normal life expectancy in ultra\u2010low risk patients. The current study highlights host\u2010related factors as the primary determinants of longevity in ET and provides a composite risk score (AAA\n                    <jats:sup>+<\/jats:sup>\n                    ) that is based on complete blood count\u2010derived parameters and host\u2010related factors. Predictive performance of the new model was shown to be superior to that of IPSET and AAA.\n                  <\/jats:p>","DOI":"10.1002\/ajh.70065","type":"journal-article","created":{"date-parts":[[2025,9,3]],"date-time":"2025-09-03T12:44:21Z","timestamp":1756903461000},"page":"2017-2027","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Triple A Plus (\n                    <scp>AAA<\/scp>\n                    <sup>+<\/sup>\n                    ) Survival Prediction Model for Essential Thrombocythemia: Analysis Involving 7308 Patients"],"prefix":"10.1002","volume":"100","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4605-3821","authenticated-orcid":false,"given":"Ayalew","family":"Tefferi","sequence":"first","affiliation":[{"name":"Division of Hematology, Department of Medicine Mayo Clinic  Rochester Minnesota 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