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Other disease features include leukocytosis, splenomegaly, thrombohemorrhagic complications, vasomotor disturbances, pruritus, and a small risk of disease progression into acute leukemia or myelofibrosis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Diagnosis:<\/jats:title><jats:p>Diagnosis is based on<jats:italic>JAK2<\/jats:italic>mutation status (PV and ET), serum erythropoietin (Epo) level (PV), and bone marrow histopathology (ET). The presence of a<jats:italic>JAK2<\/jats:italic>mutation and subnormal serum Epo level confirm a diagnosis of PV. Differential diagnosis in ET should include chronic myelogenous leukemia and prefibrotic myelofibrosis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Risk stratification:<\/jats:title><jats:p>Current risk stratification in PV and ET is designed to estimate the likelihood of thrombotic complications: high\u2010risk\u2010age &gt;60 years or presence of thrombosis history; low\u2010risk\u2010absence of both of these two risk factors. Presence of extreme thrombocytosis (platelet count &gt;1,000 \u00d7 10<jats:sup>9<\/jats:sup>\/L) might be associated with acquired von Willebrand syndrome (AvWS) and, therefore, risk of bleeding. Risk factors for shortened survival in both PV and ET include age &gt;60 years, leukocytosis, history of thrombosis, and anemia.<\/jats:p><\/jats:sec><jats:sec><jats:title>Risk\u2010adapted therapy:<\/jats:title><jats:p>Survival is near\u2010normal in ET and reasonably long in PV. The 10\u2010year risk of leukemic\/fibrotic transformation is &lt;1%\/1% in ET and &lt;5%\/10% in PV. In contrast, the risk of thrombosis exceeds 20%. The main goal of therapy is therefore to prevent thrombohemorrhagic complications and this is effectively and safely accomplished by the use of low\u2010dose aspirin (PV and ET), phlebotomy (PV), and hydroxyurea (high risk PV and ET). Treatment with busulfan or interferon\u2010\u03b1 is usually effective in hydroxyurea failures. Am. J. Hematol. 86:293\u2013301, 2011. \u00a9 2011 Wiley\u2010Liss, Inc.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ajh.21946","type":"journal-article","created":{"date-parts":[[2011,2,24]],"date-time":"2011-02-24T14:33:30Z","timestamp":1298558010000},"page":"292-301","source":"Crossref","is-referenced-by-count":30,"title":["Annual Clinical Updates in Hematological Malignancies: A Continuing Medical Education Series: Polycythemia vera and essential thrombocythemia: 2011 update on diagnosis, risk\u2010stratification, and 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