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Patients with polycythemia vera (PV) and essential thrombocythemia (ET) should be defined as high risk if age is greater than 60 years or there is a history of previous thrombosis. Risk stratification in primary myelofibrosis (PMF) should start with the International Prognostic Scoring System (IPSS) for newly diagnosed patients and dynamic IPSS for patients being seen during their disease course, with the addition of cytogenetics evaluation and transfusion status. High-risk patients with PV should be managed with phlebotomy, low-dose aspirin, and cytoreduction, with either hydroxyurea or interferon at any age. High-risk patients with ET should be managed with cytoreduction, using hydroxyurea at any age. Monitoring response in PV and ET should use the ELN clinicohematologic criteria. Corticosteroids, androgens, erythropoiesis-stimulating agents, and immunomodulators are recommended to treat anemia of PMF, whereas hydroxyurea is the first-line treatment of PMF-associated splenomegaly. Indications for splenectomy include symptomatic portal hypertension, drug-refractory painful splenomegaly, and frequent RBC transfusions. The risk of allogeneic stem-cell transplantation\u2013related complications is justified in transplantation-eligible patients whose median survival time is expected to be less than 5 years.<\/jats:p>","DOI":"10.1200\/jco.2010.31.8436","type":"journal-article","created":{"date-parts":[[2011,1,4]],"date-time":"2011-01-04T20:54:52Z","timestamp":1294174492000},"page":"761-770","source":"Crossref","is-referenced-by-count":714,"title":["Philadelphia-Negative Classical Myeloproliferative Neoplasms: Critical Concepts and Management Recommendations From European LeukemiaNet"],"prefix":"10.1200","volume":"29","author":[{"given":"Tiziano","family":"Barbui","sequence":"first","affiliation":[{"name":"From the Ospedali Riuniti di Bergamo, Bergamo; Istituto di Ricovero e Cura a Carattere Scientifico Policlinico S. 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