Polycythemia Vera (PV) and Essential Thrombocythemia (ET) are the most frequent Philadelphia chromosome-negative myeloproliferative neoplasms, the other entity being myelofibrosis. Management of patients with PV and ET is fraught with difficulties as they have an inherent tendency to cause thrombotic and hemorrhagic events. There are no curative treatment options, therefore it is important that a risk-adapted treatment approach is applied. Areas covered: This review discusses existing literature about prognosis in PV and ET, and addresses critical aspects related to defining 'high-risk' disease. In addition to the traditional risk factors such as age and prior thrombotic history, we discuss the prognostic impact of additional parameters such as cardiovascular risk factors, white blood cell count, karyotype and gene mutations. Expert commentary: We use age>60 years, presence of JAK2 mutation and a prior thrombotic history as the principle determinants of 'high-risk' for thrombosis in PV and ET, dividing the patients into very-low, low, intermediate and high-risk disease. Typically, low-risk patients are treated either with observation or aspirin alone. High-risk patients require cytoreductive therapies, along with aspirin and/or systemic anticoagulation. Intermediate-risk patients are treated on a case-by-case basis. Further, we aim to maintain a hematocrit <45% with aggressive phlebotomy in patients with PV.
Keywords: Polycythemia Vera; acute myeloid leukemia; essential thrombocythemia; high-risk; myelofibrosis; thrombosis; thrombotic risk.