Thrombotic diseases like ischemic stroke are common complications of essential thrombocythemia (ET) due to abnormal megakaryopoiesis and platelet dysfunction. Ischemic stroke in ET can occur as a result of both cerebral arterial and venous thrombosis. Management of ET is aimed at preventing vascular complications including thrombosis. Acute management of ischemic stroke in ET is the same as that in the general population without myeloproliferative disorder. However, an ET patient with ischemic stroke is at high risk for re-thrombosis and therefore additionally managed with cytoreductive therapy and antithrombotic agents. Given abnormal platelet production in ET, there is suboptimal suppression of platelets with the usual recommended dose of Aspirin for cardiovascular (CV) prevention. Hence, for optimal CV protection in ET, low dose Aspirin is recommended twice daily in an arterial thrombotic disease like atherothrombotic ischemic stroke in presence of the following risk factors: age > 60 years, Janus kinase2V617F gene mutation, presence of CV risk factors. In presence of the same risk factors, concurrent antiplatelet and anticoagulant therapy is suggested for venous thrombosis. However, increased risk of bleeding with dual anti-thrombotic agents poses a significant challenge in their use in cerebral venous thromboembolism or, atrial fibrillation in presence of the above-mentioned risk factors. We discuss these dilemmas about antithrombotic management in ischemic stroke in ET in this cased based review of literature in the light of current evidence.
© 2021 The Author(s). Published by S. Karger AG, Basel.

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