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Studies on blood eosinophils. II. Patients with Löffler's cardiomyopathy
Insights
Patients with hypereosinophilic states, like Löffler's endocarditis, show activated eosinophils. These cells release damaging products in circulation, contributing to restrictive cardiomyopathy and endomyocardial fibrosis.
Area of Science:
- Cardiology
- Hematology
- Immunology
Background:
- Hypereosinophilic states, including Löffler's endocarditis and endomyocardial fibrosis, are characterized by elevated eosinophil counts.
- These conditions can lead to severe cardiac dysfunction, specifically restrictive cardiomyopathy.
Purpose of the Study:
- To investigate the characteristics of blood eosinophils in patients with heart failure and raised eosinophil counts.
- To explore the potential role of eosinophil degranulation in the pathogenesis of cardiac damage in these patients.
Main Methods:
- Analysis of blood eosinophils from four patients with elevated eosinophil counts and heart failure.
- Cardiological assessment including identification of endocardial lesions.
- Morphological and functional characterization of patient eosinophils compared to normal controls, including receptor expression and phagocytosis assays.
Main Results:
- Three patients exhibited endocardial lesions and restrictive cardiomyopathy consistent with Löffler's endocarditis or endomyocardial fibrosis.
- All patients had >1 X 10(9) eosinophils/L with vacuolated cytoplasm and altered crystalloid granules.
- Patient eosinophils displayed IgG and C3b receptors and phagocytic activity, unlike normal eosinophils.
Conclusions:
- Circulating eosinophils in these patients exhibited characteristics of mature or stimulated cells.
- These activated eosinophils underwent degranulation in response to blood-borne stimuli.
- The findings support the hypothesis that sustained release of eosinophil products contributes to the restrictive cardiomyopathy seen in hypereosinophilic states.
Abstract:
Studies were done on blood eosinophils from four patients with raised blood eosinophil counts and heart failure. In three of the patients cardiological studies demonstrated the distinctive endocardial lesions and restrictive cardiomyopathy of Löffler's endocarditis and endomyocardial fibrosis. The fourth patient died with similar symptoms and signs. In blood films it was found that all four had more than 1 X 10(9) eosinophils per litre which were vacuolated and contained reduced numbers of crystalloid granules which were also shown to have ultrastructural changes. Unlike eosinophils from normal individuals the patients' eosinophils possessed receptors for rabbit IgG-coated erythrocytes and actively phagocytosed erythrocytes coated with rabbit IgG or human C3b. It is concluded that in these patients, a large proportion of the circulating eosinophils had developed characteristics of mature or stimulated eosinophils. This enabled them to respond to soluble substances in the bloodstream by forming endocytic vacuoles which led to degranulation of the crystalloid granules. These studies, taken in conjunction with other recent work in this field, support the concept that the restrictive cardiomyopathy of hypereosinophilic states, including Löffler's endocarditis and endomyocardial fibrosis, is a result of prolonged release of products from degranulated eosinophils while they are in the circulation.
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