A Stemi in Poems Syndrome: A Case Report

Background POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin lesions) syndrome is a rare systemic hematological disorder with multi–organ involvement. Though pathophysiology is incompletely understood, cytokine dysregulation is thought to play a central role and VEGF levels are frequently high. Case summary A 42–year–old woman with no previous cardiovascular history presented to the ED with a 3–day history of worsening intermittent and oppressive chest pain radiating to the left arm. Her father suffered from ischemic heart disease and no other conventional risk factor was known. 10 months earlier she was diagnosed with POEMS syndrome after the onset of sensory–motor polyneuropathy of the lower limbs. At the admission the patient was hemodynamically stable. ECG revealed a ST–segment elevation in the anterolateral leads and a diagnosis of STEMI was made. Transthoracic echocardiography showed a mild circumferential pericardial effusion and acute mechanical complications were promptly excluded by a cardiac CT. The patient underwent emergent coronary angiography which revealed a thrombotic sub–occlusive stenosis of the mid left anterior descending artery and no other significant coronary artery disease. PCI was performed with optimal angiographic result. Dual antiplatelet therapy with ticagrelor and acetylsalicylic acid and a high–intensity lipid–lowering treatment were started. A comprehensive echocardiography showed a mildly reduced ejection fraction of the left ventricle due to akinesia of the apex, the mid anterior and septal walls with the persistence of a minimal pericardial effusion. During hospitalization the patient remained hemodynamically stable and asymptomatic. LDL was 83 mg/dL and Lp(a) was on desirable levels. High–sensitivity troponin peak level was 9758 ng/L and subsequently decreased. Mild thrombocytosis was observed on admission and progressively normalized and VEGF was significantly increased. After optimization of the medical therapy, the patient was discharged home in good clinical condition. Discussion: The association between POEMS syndrome and acute myocardial infarction is poorly understood as myocardial ischemia is not a typical manifestation of the disease and only few cases have been reported in literature. The cytokine–mediated endothelial dysfunction and the prothrombotic mechanisms have been proposed as potential driving mechanisms in the development of coronary events in these patients.