Summary
Hypereosinophilic syndrome (HES) is a group of disorders characterized by abnormally high levels of immune cells called eosinophils in the blood (eosinophilia) and organ damage as a result of these elevated levels. Eosinophils are white blood cells that are normally produced in response to allergies, asthma, or infections. In HES, elevated levels disrupt the normal functions of organs or body systems, most commonly the skin, lungs, heart, gastrointestinal tract and nervous system. People with HES may have no symptoms or may have severe symptoms, like heart failure or stroke.
HES can be inherited or can be caused by certain bone marrow disorders, infections, or inflammatory disorders. Treatment options depend on the underlying cause and disease severity, but the overall goal of treatment is to reduce the level of eosinophils, treat any associated symptoms and prevent disease progression.
Introduction
HES was first identified as a specific syndrome in 1968. The definition for its diagnosis was established in 1975. This definition includes an eosinophil blood count greater than 1,500 cells/uL for more than 6 months, in the presence of no other identifiable cause for high blood levels of eosinophils and evidence that eosinophils are affecting organs.1 Normal eosinophil counts are less than 500 cells/uL.2
The Working Conference on Eosinophil Disorders and Syndromes introduced revised terminology for eosinophilic syndromes to improve clarity in diagnosis and classification. HES is currently categorized into five clinically relevant variants.
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