Newly Diagnosed Essential Thrombocythemia Leading to Cardiogenic Shock: A Case Report

Abstract Background Essential thrombocythemia (ET) is a myeloproliferative neoplasm characterized by uninhibited platelet production. It can present with vasomotor symptoms, and less commonly, severe thrombotic events such as myocardial infarction. ST-segment elevation myocardial infarction (STEMI) secondary to the hypercoagulable state in ET is a diagnostic challenge as the complication is rare, especially outside the typical demographics affected by ET such as the female and elderly populations. Case Presentation: A 32-year-old male presented to the emergency department with chest pain. On admission, the patient’s platelet count was 1,244,000 platelets/µL. A clinical diagnosis of STEMI was made by electrocardiogram, and the patient underwent emergent cardiac catheterization with placement of an Impella. Angiographic findings revealed a 100% flush occlusion of the ostial left anterior descending (LAD) artery and 80% occlusion of the ostial left circumflex artery (LCA) with thrombus. Successful percutaneous coronary intervention of the ostial LAD with a drug-eluting stent and balloon angioplasty of the ostial LCA was performed with full restoration of blood flow. He was initiated on aspirin and ticagrelor. The patient’s condition was complicated by cardiogenic shock for which the Impella remained in place. Subsequent transthoracic echocardiogram showed a left ventricular ejection fraction of 20–25%. Due to the patient’s markedly elevated platelet count, there was concern for possible underlying ET. A PCR assay on peripheral blood isolated from the patient was positive for a JAK2 V617F mutation. The patient was slowly weaned off the Impella over five days, and once he was hemodynamically and clinically stable, he was initiated on hydroxyurea and discharged with a wearable cardioverter defibrillator. Cardiac rehabilitation was planned. Conclusions This case illustrates the potential for severe thrombotic complications such as STEMI due to ET. Severe thrombotic complications are less common manifestations of ET in general, particularly in young males. Recognition and diagnosis of ET are critical for the institution of appropriate therapy and prevention of STEMI and cardiogenic shock among other complications.