Abnormal adhesion of red blood cells in polycythemia vera: a prothrombotic effect?

Polycythemia vera (PV) is a myeloproliferative neoplasm (MPN) characterised by the V617F activating mutation in the tyrosine kinase JAK2. PV patients exhibit increased haemoglobin levels and red cell mass because of uncontrolled proliferation of the erythroid lineage. Thrombosis and transformation to acute leukaemia are the major causes of morbidity and mortality in this disease. Increased thrombotic risk in PV patients is multifactorial and complex; it is associated with high levels of haemoglobin, impaired rheology and increased viscosity resulting from erythrocytosis. An additional parameter that might contribute to this risk was recently brought to light by work from our group showing abnormal activation of adhesion proteins in PV RBCs. In this review we provide an overview of these recent findings and discuss how the pro-adhesive features of JAK2V617F-positive red blood cells might initiate and contribute to the circulatory complications described in PV.