November 22, 2023
Giulia Benevolo,1 Monia Marchetti,2 Remo Melchio,3 Eloise Beggiato,1 Chiara Sartori,4 Carlo Alberto Biolé,5 Davide Rapezzi,6 Benedetto Bruno,1,7 Alberto Milan8
1University Hematology Division, Città della Salute e della Scienza di Torino, Turin, Italy; 2Hematology and Transplant Unit, Azienda Ospedaliera SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy; 3Division of Internal Medicine, A.O. S. Croce E Carle, Cuneo, Italy; 4Cardiology, Azienda Ospedaliera SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy; 5SCDO Cardiology, AOU San Luigi Gonzaga Orbassano, Turin, Italy; 6Hematology Division A.O. S. Croce e Carle, Cuneo, Italy; 7Department of Molecular Biotechnolgies and Medical Sciences, University of Turin, Turin, Italy; 8Department of Medical Sciences, University of Turin, Città della Salute e della Scienza di Torino, Turin, Italy
Correspondence: Giulia Benevolo, University Hematology Division, Città della Salute e della Scienza di Torino, via Genova 3, Turin, 10126, Italy, Tel +39 011 633 4301, Fax +39 011 633 4187, Email gbenevolo@cittadellasalute.to.it
Abstract: Polycythemia vera (PV) is a myeloproliferative neoplasm characterized by aberrant myeloid lineage hematopoiesis with excessive red blood cell and pro-inflammatory cytokine production. Patients with PV present with a range of thrombotic and hemorrhagic symptoms that affect quality of life and reduce overall survival expectancy. Thrombotic events, transformation into acute myeloid leukemia, and myelofibrosis are largely responsible for the observed mortality. Treatment of PV is thus primarily focused on symptom control and survival extension through the prevention of thrombosis and leukemic transformation. Patients with PV frequently experience thrombotic events and have elevated cardiovascular risk, including hypertension, dyslipidemias, obesity, and smoking, all of which negatively affect survival. To reduce the risk of thrombotic complications, PV therapy should aim to normalize hemoglobin, hematocrit, and leukocytosis and, in addition, identify and modify cardiovascular risk factors. Herein, we review what is currently known about the associated cardiovascular risk and propose strategies for diagnosing and managing patients with PV.
Plain Language Summary: Patients with the myeloproliferative neoplasm (MPN) polycythemia vera (PV) are at increased risk of cardiovascular (CV) events, including stroke, heart attacks, and peripheral arterial disease. High blood pressure, smoking, and dyslipidemia are common in MPN and contribute to the increased cardiovascular risk. Effectively controlling cardiovascular risk factors in PV, along with appropriate hematological therapy such as direct-acting oral anticoagulants alone or in combination with aspirin, may improve the outcomes of patients with PV, but further research is needed.