Cardiac Localization of Eosinophil-Granule Major Basic Protein in Acute Necrotizing Myocarditis

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Abstract
THE association of eosinophilia and endomyocarditis has been well documented. A form of Löffler's endocarditis,1 characterized by eosinophilic pancarditis, muscle necrosis, and arteritis, is usually a feature of certain systemic diseases, including the hypereosinophilic syndrome,2 3 4 eosinophilic leukemia,5 polyarteritis nodosa,6 , 7 drug-induced hypersensitivity,8 bronchial asthma,9 10 11 biliary tract carcinoma,12 and parasitic disease.13 The cardiac involvement is characterized by a subacute course and symptoms of congestive heart failure. Treatment with glucocorticoids and cytotoxic agents is effective.14 Fulminant acute necrotizing eosinophilic myocarditis occurring in the absence of notable extracardiac disease has been reported only rarely.15 The disease has a short course and is rapidly fatal. Glucocorticoids have not been found to be effective in the acute form of the disease, perhaps because in the few instances when glucocorticoids were used, they were introduced late in the clinical course.15 We describe two children who had sudden cardiac deterioration after a short illness, presumably viral, and died within a few hours of fulminant acute necrotizing eosinophilic myocarditis. The cardiac decompensation was accompanied by a pattern of infarction on electrocardiography and, in one patient, by peripheral eosinophilia. In both patients, deposition of the major basic protein of the eosinophil granule was identified within necrotic myocardium, and in one patient, abnormalities of eosinophil granules characteristic of an activated state were seen on electron microscopy. Extracardiac disease at autopsy was largely related to cardiac failure.