Idiopathic hypereosinophilia with cardiac involvement

摘要

HISTORY AND CLINICAL FINDINGS: A 34-year-old previously healthy woman was admitted to another hospital because of abdominal pain, cough and dyspnea. Peripheral eosinophilia was present. Two months later she was admitted to the cardiology department with signs of mitral regurgitation. Dyspnea, fatigue, skin rashes with pruritus and a systolic murmur were noted. INVESTIGATIONS:: Laboratory tests showed 11,4l leukocytes (normal range 4,8 - 10,8l) with an eosinophilia of 19 % (normal range < 4 %) corresponding to 2,2l. Cardiac magnetic resonance imaging revealed endomyocardial fibrosis involving the posterior mitral leaflet with resulting valvular regurgitation. Doppler ultrasound showed restrictive heart failure. DIAGNOSIS, THERAPY AND FURTHER COURSE: The diagnosis of idiopathic hypereosinophilia most likely as part of hypereosinophilic syndrome with cardiac involvement was made. The patient was treated with digitalis, diuretics and peptidyl dipeptidase (PDP) inhibitor. The treatment with glucocorticoids and cytotoxic agent to achieve a reduction of eosinophil count was ended by the patient a few weeks later. CONCLUSION: The hypereosinophilic syndrome with endomyocardial fibrosis is rare, and its prognosis is grave. The pathophysiological mechanisms are not entirely clear, nearly 70 years after Loffler first described fibrous endocarditis with eosinophilia. Patients receive symptomatical medical therapies. Additional surgical treatment has been reported,. Antihypereosinophilic therapy is used to control the disease.