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The Idiopathic Hypereosinophilic Syndrome
Clinical, Pathophysiologic, and Therapeutic Considerations
- ANTHONY S. FAUCI, M.D.;
- JOHN B. HARLEY, M.D., Ph.D.;
- WILLIAM C. ROBERTS, M.D.;
- VICTOR J. FERRANS, M.D., Ph.D.;
- HARVEY R. GRALNICK, M.D.; and
- BARBARA H. BJORNSON, M.D.
Abstract
The idiopathic hypereosinophilic syndrome (HES) represents a heterogeneous group of disorders with the common features of prolonged eosinophilia of an undetectable cause and organ system dysfunction. Fifty patients with the idiopathic HES were studied over 11 years at the National Institutes of Health. Multiple organ systems were involved; bone marrow hypereosinophilia was common to all patients, but the most severe clinicopathologic involvement was of the heart and nervous system. Postmortem gross pathologic examination of the hearts of patients with idiopathic and nonidiopathic HES suggested that the common mechanism of cardiac disease is the eosinophilia. Endomyocardial biopsy findings showed that the endothelial cells in the endocardium and of the microvasculature were the primary targets of the tissue damage. This damage initiates thrombosis; endocardial fibrosis and restrictive endomyocardopathy may follow. Invitro culture of circulating eosinophil colony-forming units showed some normal studies, some studies showing increased progenitor cells committed to eosinophil development,and others showing an excess production of eosinophil colony-stimulating factor. Chemotherapy to lower the eosinophil counts has resulted in marked improvement of HES prognosis, as have agressive medical and surgical approaches to cardiovascular complications.
Article and Author Information
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▸ An edited transcription of a Clinical Staff Conference at the Clinical Center, Bethesda, Maryland, 12 February 1981, sponsored by the National Institute of Allergy and Infectious Diseases, National Institute of Health, U.S. Department of Health and Human Services.
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▸ Authors who wish to cite a section of this conference and specifically indicate its author can use this example for the form of reference:
HARLEY JB. Clinical manifestations of patients with hypereosinophilic syndrome, pp. 82-4. In: FAUCI AS, moderator. The idiopathic hypereosinophilic syndrome: clinical, pathophysiologic, and therapeutic considerations. Ann Intern Med. 1982;97:78-92.
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▸ Requests for reprints should be addressed to Dr. Anthony S. Fauci; Building 10, Room 11B09, National Institutes of Health, Bethesda, MD 20205.
