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Prognostically adverse mutations include\n                      <jats:italic>SRSF2, IDH2, RUNX1<\/jats:italic>\n                      , and\n                      <jats:italic>U2AF1<\/jats:italic>\n                      , with a combined incidence of 5%\u201310%.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Survival and Prognosis<\/jats:title>\n                    <jats:p>Median survival is \u205315\u2009years but exceeds 35\u2009years for patients aged \u226440\u2009years. Risk factors for survival include older age, leukocytosis, abnormal karyotype, and the presence of adverse mutations. Twenty\u2010year risk for thrombosis, post\u2010PV MF, or AML are \u205326%, 16% and 4%, respectively.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Risk Factors for Thrombosis<\/jats:title>\n                    <jats:p>\n                      Two risk categories are considered: high (age &gt;60\u2009years or thrombosis history) and low (absence of both risk factors). Additional predictors for arterial thrombosis include cardiovascular risk factors and for venous thrombosis higher absolute neutrophil count and\n                      <jats:italic>JAK2<\/jats:italic>\n                      V617F allele burden.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Treatment<\/jats:title>\n                    <jats:p>Current goal of therapy is to prevent thrombosis. Periodic phlebotomy, with a hematocrit target of &lt;45%, combined with once\u2010 or twice\u2010daily aspirin (81\u2009mg) therapy, absent contraindications, is the backbone of treatment in all patients, regardless of risk category. Cytoreductive therapy is reserved for high\u2010risk disease with first\u2010line drugs of choice being hydroxyurea and pegylated interferon\u2010\u03b1 and second\u2010line busulfan and ruxolitinib. In addition, systemic anticoagulation is advised in patients with venous thrombosis history.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Additional Treatment Considerations<\/jats:title>\n                    <jats:p>\n                      At the present time, we do not consider a drug\u2010induced reduction in\n                      <jats:italic>JAK2<\/jats:italic>\n                      V617F allele burden, which is often incomplete and seen not only with peg\u2010IFN but also with ruxolitinib and busulfan, as an indicator of disease\u2010modifying activity, unless accompanied by cytogenetic and independently\u2010verified morphologic remission. Accordingly, we do not use the specific parameter to influence treatment choices. The current review also includes specific treatment strategies in the context of pregnancy, splanchnic vein thrombosis, pruritus, perioperative care, and post\u2010PV MF.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ajh.27002","type":"journal-article","created":{"date-parts":[[2023,6,26]],"date-time":"2023-06-26T05:54:37Z","timestamp":1687758877000},"page":"1465-1487","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":231,"title":["Polycythemia vera: 2024 update on diagnosis, risk\u2010stratification, and management"],"prefix":"10.1002","volume":"98","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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