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However, the same molecular markers might also be present in prefibrotic myelofibrosis, whose morphological distinction from ET is prognostically relevant.<\/jats:p><\/jats:sec><jats:sec><jats:title>Survival and leukemic\/fibrotic transformation<\/jats:title><jats:p>Median survivals are approximately 14 years for PV and 20 years for ET; the corresponding values for younger patients (age &lt;60 years) are 24 and 33 years. Life\u2010expectancy in ET is inferior to the control population. Driver mutational status has not been shown to affect survival in ET whereas the presence of<jats:italic>JAK2\/MPL<\/jats:italic>mutations has been associated with higher risk of arterial thrombosis and that of<jats:italic>MPL<\/jats:italic>with higher risk of fibrotic progression. Risk factors for overall survival in both ET and PV include advanced age, leukocytosis and thrombosis. Leukemic transformation rates at 20 years are estimated at &lt;10% for PV and 5% for ET; fibrotic transformation rates are slightly higher. Most recently,<jats:italic>ASXL1<\/jats:italic>,<jats:italic>SRSF2<\/jats:italic>, and<jats:italic>IDH2<\/jats:italic>mutations have been associated with inferior overall, leukemia\u2010free or fibrosis\u2010free survival in PV; similarly adverse mutations in ET included<jats:italic>SH2B3<\/jats:italic>,<jats:italic>SF3B1<\/jats:italic>,<jats:italic>U2AF1<\/jats:italic>,<jats:italic>TP53<\/jats:italic>,<jats:italic>IDH2<\/jats:italic>, and<jats:italic>EZH2<\/jats:italic>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Thrombosis risk stratification<\/jats:title><jats:p>Current risk stratification in PV and ET is designed to estimate the likelihood of recurrent thrombosis. Accordingly, PV includes two risk categories: high\u2010risk (age &gt;60 years or thrombosis history) and low\u2010risk (absence of both risk factors). In ET, risk stratification includes four categories: very low risk (age \u226460 years, no thrombosis history,<jats:italic>JAK2\/MPL<\/jats:italic>un\u2010mutated), low risk (age \u226460 years, no thrombosis history,<jats:italic>JAK2\/MPL<\/jats:italic>mutated), intermediate risk (age &gt;60 years, no thrombosis history,<jats:italic>JAK2\/MPL<\/jats:italic>un\u2010mutated), and high risk (thrombosis history<jats:styled-content>or<\/jats:styled-content>age &gt;60 years with<jats:italic>JAK2\/MPL<\/jats:italic>mutation). In addition, presence of extreme thrombocytosis (platelets &gt;1000 \u00d7 10(9)\/L) might be associated with acquired von Willebrand syndrome (AvWS) and, therefore, risk of bleeding.<\/jats:p><\/jats:sec><jats:sec><jats:title>Risk\u2010adapted therapy<\/jats:title><jats:p>The main goal of therapy in PV and ET is to prevent thrombohemorrhagic complications. All patients with PV require phlebotomy to keep hematocrit below 45% and once\u2010daily aspirin (81 mg). In addition, high\u2010risk patients with PV require cytoreductive therapy. Very low risk ET patients might not require any form of therapy while low\u2010risk patients require at least once\u2010daily aspirin therapy. Cytoreductive therapy is also recommended for high\u2010risk ET patients but it is not mandatory for intermediate\u2010risk patients. First\u2010line drug of choice for cytoreductive therapy, in both ET and PV, is hydroxyurea and second\u2010line drugs of choice are interferon\u2010\u03b1 and busulfan. We currently do not recommend treatment with ruxolutinib or other<jats:italic>JAK2<\/jats:italic>inhibitors in PV or ET, unless in the presence of severe and protracted pruritus or marked splenomegaly that is not responding to the aforementioned drugs. Screening for AvWS is recommended before administrating aspirin, in the presence of extreme thrombocytosis. Am. J. 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