Acute Coronary Syndrome in Essential Thrombocythemia and Polycythemia Vera

Essential thrombocythemia (ET) and polycythemia vera (PV) are rare chronic myeloproliferative neoplasms that have hemostatic complications (i.e., thrombosis and hemorrhage), leading to morbidity and mortality. Treatment in ET or PV is directed toward preventing thrombotic consequences. However, the increased risk of bleeding may complicate antithrombotic therapy in these patients. Thrombosis may occur in both arterial and venous vascular beds. Arterial thrombosis, such as acute coronary syndrome (ACS), is more frequent than venous thrombosis. Both ET and PV can initially present as an ACS, the management of which is highly challenging in the absence of clinical guidelines that are dedicated to such patients. The management of ACS in patients with ET or PV has not been well described in literature. Herein, this review discusses the pathogenesis, characteristics, and management of ACS in patients with ET or PV.