Fatal Loeffler's endocarditis due to hypereosinophilic syndrome

Abstract

Hypereosinophilic syndrome (HES) is a rare disorder that can manifest in various organ systems. We report the case of a 54-year-old woman with a remote history of seizure disorder who presented with early signs of right-sided heart failure. Laboratory studies showed significant eosinophilia (8 × 10 9 l-1). Computed tomography showed heterogeneity of the liver, mild ascites, moderate pleural effusion, multiple small pulmonary emboli, and a large right ventricular mass. Cardiac magnetic resonance imaging demonstrated that the right ventricular mass was due to thrombus and extensive endomyocardial fibrosis, consistent with Loeffler's endocarditis. Bone marrow biopsy showed marked eosinophilia but no abnormal myeloid maturation or a lymphoproliferative disorder; flow cytometry showed no clonality. Extensive infectious, immunologic, and toxicological studies were negative. Despite resolution of peripheral eosinophilia with medical management, including corticosteroids and cytotoxic agents, anticoagulation for pulmonary emboli and ventricular thrombus, and conventional treatment for heart failure, she developed worsening anasarca and died from ventricular fibrillation within 4 weeks of presentation. Autopsy confirmed the diagnosis. Loeffler's endocarditis, usually a late manifestation of HES, is characterized by fibrous thickening of the endocardium, leading to apical obliteration and restrictive cardiomyopathy, resulting in heart failure, thromboembolic events, or atrial fibrillation. HES is a potentially fatal disease with less than 50% reported 10-year survival. This case presentation is unusual in its rapidly progressive course leading to sudden death. © 2007 Wiley-Liss, Inc.