Cerebral Thrombosis and Myeloproliferative Neoplasms

Andrea Artoni, Paolo Bucciarelli, Ida Martinelli

Myeloproliferative neoplasms (MPN) are acquired clonal disorders characterized by the proliferation of bone marrow myeloid cells. Different somatic mutations have been recently associated with MPN, the most common being JAK-2 V617F. Among MPN, polycythemia vera and essential thrombocythemia are particularly associated with an increased risk to develop thrombotic complications, either arterial or venous. Cerebrovascular events (stroke and transient ischemic attacks) are prevalent, accounting for approximately two-thirds of all events. Also cerebral vein thrombosis can complicate MPN and can be the first manifestation of the disease. Risk factors for thrombosis in patients with MPN are related or unrelated to the disease. Among the former there are cellular risk factors, such as increased white blood cell counts, vascular cell activation, endothelial dysfunction, and plasmatic risk factors, such as increased plasma viscosity, reduced levels of protein S, increased thrombin generation. The latter include increased age and previous thrombotic events. In addition, common cardiovascular risk factors (smoking, hypertension, diabetes, dyslipidemia, obesity) contribute to the pathogenesis of arterial events, whereas circumstantial risk factors (particularly oral contraceptive use and pregnancy/puerperium) to that of venous events. Primary prevention of arterial thrombosis with antiplatelet therapy is warranted in the majority of patients with MPN, whereas primary prevention of venous thrombosis is limited to anticoagulant prophylaxis during high-risk situations. Secondary prevention includes long-term antiplatelet therapy for arterial and short- or long-term anticoagulant therapy for venous thrombosis, depending on the risk factors present at the first event.