{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T11:05:02Z","timestamp":1792062302778,"version":"4.4.0"},"reference-count":90,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2018,11,9]],"date-time":"2018-11-09T00:00:00Z","timestamp":1541721600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2019,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>\n<jats:bold>Disease Overview:<\/jats:bold> Polycythemia vera (PV) and essential thrombocythemia (ET) are myeloproliferative neoplasms respectively characterized by erythrocytosis and thrombocytosis; other disease features include leukocytosis, splenomegaly, thrombosis, bleeding, microcirculatory symptoms, pruritus, and risk of leukemic or fibrotic transformation.<\/jats:p><jats:p>\n<jats:bold>Diagnosis:<\/jats:bold> Bone marrow morphology remains the cornerstone of diagnosis. In addition, the presence of <jats:italic>JAK2<\/jats:italic> mutation is expected in PV while approximately 90% of patients with ET express mutually exclusive <jats:italic>JAK2<\/jats:italic>, <jats:italic>CALR,<\/jats:italic> or <jats:bold>m<\/jats:bold>yeloproliferative leukemia mutations. In ET, it is most important to exclude the possibility of prefibrotic myelofibrosis.<\/jats:p><jats:p>\n<jats:bold>Survival<\/jats:bold>: Median survivals are 14 years for PV and 20 years for ET; the corresponding values for younger patients are 24 and 33 years. Certain mutations (mostly spliceosome) and abnormal karyotype might compromise survival in PV and ET. Life\u2010expectancy in ET is inferior to the control population. Driver mutations have not been shown to affect survival in ET. Risk of thrombosis is higher in <jats:italic>JAK2\u2010<\/jats:italic>mutated ET. Leukemic transformation rates at 10 years are estimated at &lt;1% for ET and 3% for PV.<\/jats:p><jats:p>\n<jats:bold>Thrombosis Risk:<\/jats:bold> In PV, 2 risk categories are considered: high (age &gt; 60 years or thrombosis history present) and low (absence of both risk factors); in ET, 4 risk categories are considered: very low (age \u2264 60 years, no thrombosis history, <jats:italic>JAK2<\/jats:italic> wild\u2010type), low (same as very low but <jats:italic>JAK2<\/jats:italic> mutation present), intermediate (age &gt; 60 years, no thrombosis history, <jats:italic>JAK2<\/jats:italic> wild\u2010type) and high (thrombosis history present <jats:italic>or<\/jats:italic> age &gt; 60 years with <jats:italic>JAK2<\/jats:italic> mutation).<\/jats:p><jats:p>\n<jats:bold>Risk\u2010Adapted Therapy:<\/jats:bold> The main goal of therapy in both PV and ET is to prevent thrombohemorrhagic complications. All patients with PV require phlebotomy to keep hematocrit below 45% and once\u2010 or twice\u2010daily aspirin (81 mg), in the absence of contraindications. Very low\u2010risk ET might not require therapy while aspirin therapy is advised for low\u2010risk disease. Cytoreductive therapy is recommended for high\u2010risk ET and PV but it is not mandatory for intermediate\u2010risk ET. 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 do not recommend treatment with ruxolutinib in PV, unless in the presence of severe and protracted pruritus or marked splenomegaly that is not responding to the aforementioned drugs.<\/jats:p>","DOI":"10.1002\/ajh.25303","type":"journal-article","created":{"date-parts":[[2018,10,5]],"date-time":"2018-10-05T15:01:49Z","timestamp":1538751709000},"page":"133-143","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":195,"title":["Polycythemia vera and essential thrombocythemia: 2019 update on diagnosis, risk\u2010stratification and management"],"prefix":"10.1002","volume":"94","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 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