Polycythaemia vera

Abstract

Polycythaemia vera (PV) is a clonal stem cell disorder characterized by erythrocytosis and associated with burdensome symptoms, risk of thrombohaemorrhagic complications, and transformation to myelofibrosis and acute myeloid leukaemia. Diagnostic criteria are very recently revised by the World Health Organization (WHO) based on haemoglobin and haematocrit levels, bone marrow morphology consistent with trilineage proliferation and presence of the JAK2 V617 mutation. Cytoreductive therapy is indicated in patients at increased risk of thrombosis. Hydroxyurea (HU) remains the most commonly used first-line cytoreductive therapy and interferon (IFN) is used either at failure of HU or in selected patients as first-line therapy. A recent phase 3 trial has shown the superiority of the JAK1/2 inhibitor ruxolitinib in comparison to best available treatment in HU-intolerant or resistant patients.