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 x 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.

Hypereosinophilic Syndrome Presenting with Large Left Ventricular Apical Thrombus and Pulmonary Embolism

“…Pulmonary symptoms may be due to primary eosinophilic infiltration of lung parenchyma, congestive heart failure or embolus originating from the right ventricle 6 . Cases with PE are commonly reported with right ventricular involvement or with deep vein thrombosis 7,8 . Interestingly, our patient had multiple PEs without any source.…”

“…6 Cases with PE are commonly reported with right ventricular involvement or with deep vein thrombosis. 7,8 Interestingly, our patient had multiple PEs without any source. Clinically and radiologically detected "thrombus," may not be therefore only a true thrombus but also may contain bulks of eosinophils as also previously suggested.…”

Loeffler endocarditis: What have we learned?

“…This novel agent has been proposed as a candidate to become the first line treatment in Loeffler endocarditis, especially when certain gene mutations are present [19]. It is important to note that despite achieving a complete resolution of the hyperosinophilia, the prognosis could be still poor, with some cases developing serious complications (even death), as described in this issue [14]. By treating the eosinophilia it is possible to halt the progression of the disease, but the damage already done may be enough to induce lethal consequences.…”

“…The symptoms can be later worsened by the development of systolic dysfunction through ventricular remodeling [13]. In the case published in this issue of the American Journal of Hematology by Chao et al [14], the authors describe an unusual, rapidly progressing presentation of this entity. Other manifestations may include tachyarrhythmias (supraventricular or ventricular) and pulmonary or systemic embolization [4].…”

Löffler’s Endocarditis: First Report of Successful Mitral and Tricuspid Valve Replacements in a Patient with Long-Standing Hypereosinophilia

“…5 Estimated 10-year survival is less than 50% in patients with HES and less than 30% in those with endomyocardial fibrosis. 6 Some 60% of patients with HES tend to have cardiac involvement, with endomyocardial fibrosis being the main finding, sometimes with valve leaflet vegetations leading to progressive scarring and restricted valve movement. 7 Classic history and physical signs are highlighted in Table 1.…”