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Cardiomyopathy in a child with hypereosinophilic syndrome
Insights
This case study details a fatal pediatric case of hypereosinophilic syndrome (HES), a rare condition characterized by high eosinophil counts. The study highlights the critical cardiac involvement and potential association with leukemia in children with HES.
Area of Science:
- Pediatric Cardiology
- Hematology
- Immunology
Background:
- Hypereosinophilic syndrome (HES) is a rare disorder characterized by persistent, marked eosinophilia.
- Pediatric HES is less common than in adults, with varied clinical presentations.
- Understanding the etiopathogenesis and clinical course is crucial for early diagnosis and management.
Observation:
- A 9-year-old boy presented with severe constitutional symptoms including fatigue, anorexia, weight loss, and fever.
- Marked absolute eosinophilia (48,000/microL) was a key initial finding.
- Pulmonary infiltrates, mitral regurgitation, and heart failure developed sequentially, indicating significant systemic involvement.
Findings:
- The patient experienced rapid disease progression, succumbing to heart failure within 9 months.
- Necropsy revealed cardiomegaly, biventricular subendocardial fibrosis, and intracardiac thrombi.
- Review of 12 pediatric cases suggests a strong association between HES and leukemia in children, with half of cases linked to hematologic malignancies.
Implications:
- This case underscores the severe cardiac manifestations of pediatric HES and the importance of cardiac evaluation in affected children.
- The findings emphasize the need for prompt diagnosis and aggressive management strategies for HES in pediatric populations.
- The potential link to leukemia necessitates thorough hematologic assessment in pediatric HES cases to guide treatment and prognosis.
Abstract:
A 9-year-old boy presented with increasing fatigue, anorexia, weight loss, fever, and absolute eosinophilia (48,000/microL). Pulmonary infiltrates occurred 3 months later. A murmur of mitral regurgitation was heard 5 months after onset of illness, and heart failure soon followed. Despite corticosteroid therapy the eosinophilia persisted intermittently until 1 month before death. The patient died within 9 months of the onset of illness. At necropsy there was cardiomegaly with subendocardial fibrosis in the right and left ventricles. Thrombi were present in the left ventricular apex and behind the posterior mitral leaflet. The findings in 12 previously reported pediatric cases are reviewed. The etiopathogenesis of the hypereosinophilic syndrome is discussed: half of the cases in children are associated with leukemia.