MYELOPROLIFERATIVE HYPEREOSINOPHILIC SYNDROME A RARE CAUSE OF DILATED CARDIOMYOPATHY WITH A REVIEW

Abstract

Hypereosinophilic syndrome (HES) is aheterogenous group of rare haematological disorders, the majority of which are idiopathic. Cardiac manifestations of HES are rare and the myeloproliferative HES (M-HES) is very rare. Eosinophilic myocarditis and endomyocardial fibrosis are a typical course of morbidity and mortality in hypereosinophilic syndrome. We present a case of idiopathic eosinophilia who presented with dyspnea, abdominal distention and edema with peripheral eosinophilia (82%) in a complete blood count test done before presentation to us. Echocardiography showed enlarged cardiac chambers, global hypokinesia,moderate to severe tricuspid regurgitation, with reduced left ventricular ejection fraction <40% suggestive of dilated cardiomyopathy (DCM). Parasitic disease, autoimmune disease and drug induced cardiomyopathy were excluded as possibilities. He was treated with steroids, imatinib, and anti failure therapy. Cardiac function improved with therapy and the eosinophilic count reduced. Monitoring chest radiography and electrocardiogram and echocardiography according to fluctuations in eosinophils may enable early detection and treatment of cardiac involvement in patients with HES.