Abstract
Polycythemia vera (PV) is a chronic Philadelphia-negative myeloproliferative neoplasm caused by JAK2 mutation and characterized predominantly by the overproduction of red blood cells. The current treatment strategies of PV are based on periodic phlebotomies aimed at preventing thrombotic events associated with increased hematocrit levels. Additional therapies to mitigate the thrombotic burden, which represents the most important predictor of reduced survival in PV patients, include cytoreductive therapies, low-dose aspirin, and systemic anticoagulation. This concise review summarizes the current knowledge on the management of the thrombotic risk in PV patients.