ACUTE MYOCARDIAL INFARCTION: AN UNUSUAL INITIAL PRESENTATION OF POLYCYTHEMIA VERA

Abstract

TOPIC: Critical Care TYPE: Medical Student/Resident Case Reports INTRODUCTION: Polycythemia Vera (PV) is a chronic myeloproliferative disorder that affects the hematopoietic progenitor cells and increases the production of red cells, platelets and granulocytes1. The incidence of PV is around 2.3 per 100,000 persons/year2. Major cause of morbidity and mortality in PV patients is thrombosis that is either microcirculatory or arterial4. Hyperviscosity and thrombocytosis lead to coronary thrombosis resulting in acute myocardial infarction (AMI)4. However, the initial presentation of PV as AMI is extremely rare, with less than 10 cases reported in literature4. This is a unique case of a young healthy male with no cardiovascular risk factors, who presented with an AMI with the ultimate diagnosis of PV. CASE PRESENTATION: A 42-year-old French male presented with severe substernal chest pain radiating to the left arm, nausea, diaphoresis and left arm numbness ongoing for an hour. He had no significant medical, social or family history. He had a HR of 101 BPM and a BP of 137/92 mmHg. Labs included: high sensitivity troponin 701 pg/mL, LDL 111mg/dL, WBC 11.7K, platelets 357K, Hgb 22.5 g/dL and Hct 67.6%. An EKG was suggestive of an antero-septal STEMI. A cardiac catheterization with a drug eluting stent was deployed in the left anterior descending artery that was 99% occluded. The findings of an elevated hemoglobin and hematocrit triggered a cascade of investigations. Erythropoietin level was unremarkable, while bone marrow biopsy displayed panmyelosis. Imaging demonstrated hepatosplenomegaly. Ultimately, the diagnosis of PV was established when genetic testing revealed a JAK2 V617F mutation. Post-MI and PV treatment with hydroxyurea and therapeutic phlebotomies to maintain a hematocrit goal of less than 45% was initiated. DISCUSSION: Coronary events in PV patients occur at a rate of 11.4% in 10-year follow-up4. The two most important risk factors for thrombosis are advanced age and prior history of thrombosis1. Although not clearly established, overproduction of thromboxane A2, endothelial dysfunction, platelet and leukocyte activation are believed to contribute to vascular events4. The mainstay of treatment of PV is antiplatelet medications, therapeutic phlebotomies and cytoreductive drugs4. There are no current guidelines for risk reduction of stent restenosis or the treatment of AMI in PV1. CONCLUSIONS: This case emphasizes the need to consider PV in the differential diagnosis of acute coronary syndrome in young patients without any cardiovascular risk factors. Furthermore, it re-affirms the need for an annual examination to identify abnormal studies and the benefit of timely intervention. REFERENCE #1: Adel G. Polycythemia Vera and Acute Coronary Syndromes: Pathogenesis, Risk Factors and Treatment. J Hematol Thromboembolic Dis. 2013;01(01). doi:10.4172/2329-8790.1000107 REFERENCE #2: Bahbahani H, Aljenaee K, Bella A. Polycythemia vera presenting as acute myocardial infarction: An unusual presentation. J Saudi Hear Assoc. 2015;27(1):57-60. doi:10.1016/j.jsha.2014.07.003 REFERENCE #3: Tefferi A, Rumi E, Finazzi G, et al. Survival and prognosis among 1545 patients with contemporary polycythemia vera: An international study. Leukemia. 2013;27(9):1874-1881. doi:10.1038/leu.2013.163 DISCLOSURES: No relevant relationships by Madeeha Banu, source=Web Response No relevant relationships by Rohan Bodapati, source=Web Response No relevant relationships by palak desai, source=Web Response No relevant relationships by Brendan Gilmore, source=Web Response No relevant relationships by Kenneth Joseph, source=Web Response No relevant relationships by nitish singh nandu, source=Web Response No relevant relationships by Swetha Paduri, source=Web Response No relevant relationships by Nehal Patel, source=Web Response