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Introduction: Acute coronary syndromes (ACS) mostly occur in patients with traditional risk factors. Especially in young adults without major cardiovascular (CV) risk factors, one of the less common causes of ACS is myeloproliferative neoplasms (MPNs)., Methods: We retrospectively collected data on 11 consecutive patients (nine men, two women, mean age 40.18±8.4 years) with a diagnosis of MPN who presented with ACS. The demographic characteristics of the study population, type of MPN, clinical manifestations, location of myocardial infarction (MI), coronary angiography findings, complete blood count and other related findings, and treatment strategy before and after diagnosis were analyzed., Results: Six patients were diagnosed with polycythemia vera, four with essential thrombocytosis and one with primary myelofibrosis. A JAK2 mutation was found in nine patients. Mean time to diagnosis of MPN was 2.81 years after presenting ACS and mean age at first MI was 32.9±6 years. Six patients had no major CV risk factors. Ten patients had anterior MI and one had inferior MI. After initiation of specific treatment for MPN, no recurrent thrombotic events were observed in a mean follow-up of 4±2.44 years., Conclusions: In young adults presenting with ACS, MPNs should be considered, especially in the absence of atherosclerotic coronary artery lesions. It is also important to pay attention to blood cell count abnormalities seen in intracoronary thrombotic events. Early diagnosis and treatment of MPNs is essential to prevent recurrence of thrombotic events and may reduce mortality and morbidity related to thrombotic complications., (Copyright © 2019 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.)