Polycythemia vera (PV) is classified by the World Health Organization (WHO) under the BCR-ABL-negative myeloproliferative neoplasms (MPNs) and is characterised by clonal proliferation of myeloid cells, which leads primarily to an increased red blood cell mass. Bone marrow morphology remains the cornerstone of diagnosis. Patients can present with thrombosis, microcirculatory symptoms, haemorrhage, splenomegaly, pruritus and other symptoms that reduce their quality of life and they are at risk of transformation to secondary myelofibrosis (MF) or acute myeloid leukaemia (AML). The main goal of therapy in PV is to minimise the thrombotic risk. To achieve this goal PV patients are being treated with low-dose aspirin and phlebotomies to reach a target haematocrit below 45%. In addition, high-risk patients are being treated with cytoreductive agents. Over the last years, new insights in the pathophysiology, diagnosis and prognosis of polycythemia vera were acquired and novel therapeutic options are available. In this paper we give an update on PV and provide diagnostic and therapeutic recommendations, taking into account the Belgian situation.
(BELG J HEMATOL 2021;12(6):258-74)