Resumo

• Generally a disease of middle and older age.
• Carries increased risks of thrombosis, haemorrhage, myelofibrosis, and acute leukaemia.
• Diagnosis is strongly associated with the presence of the JAK2V617F mutation, although this mutation is not specific for polycythaemia vera.
• Treatment is based on stratification for risk of thrombosis, but includes low-dose aspirin and phlebotomy for most patients. High-risk patients also receive cytoreductive therapy.
• Ongoing and future studies will be required to further define disease aetiology and appropriate therapy.