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Antiphospholipid antibody syndrome (APS) is a disorder associated with both arterial and venous thromboembolic disease, including acute myocardial infarction (AMI). Given that management with anticoagulants is critical and differs from usual AMI care, identification of key discriminators of patients with AMI with APS is important.We performed an English-language systematic review of the literature of cases and case series of patients with AMI and APS from inception until 20 March 2016, collecting demographics, investigations, and outcomes.Forty cases of AMI because of APS were identified from 27 articles. Patients were younger than typical AMI patients (41.10±13.61 years) and 45% were women. STelevation myocardial infarction was the presentation in 45% (18/40) of cases. The average platelet count was 130 000±138 912 c/mm in the 10 cases reporting it and partial thromboplastin time was elevated in all four reporting it. Coronary arteries were described as normal or with acute thrombosis in 75%. Three died during hospitalization and six had recurrence of myocardial infarction within 3 months after admission.APS should be considered in young patients with AMI, especially if previous unprovoked thromboses, lower platelet counts, high partial thromboplastin times, and normal coronary arteries or coronary thromboses are identified.