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Hypereosinophilic Syndrome
The hypereosinophilic syndrome (HES) comprises a group of rare leukoproliferative disorders characterized by prolonged and sustained peripheral blood- and tissue eosinophilia leading to end-organ damage.1-3
HES is clinically heterogeneous, but has 3 hallmark features:3
- Sustained eosinophilia (total eosinophil count >1500/µL for >6 months)
- Absence of apparent etiologies for eosinophilia, including parasitic infections and allergic diseases
- Signs and symptoms of organ involvement
Epidemiology
The exact incidence of HES is unknown. According to 1 estimate, 1 or 2 new cases can be expected per year per 200,000 individuals.2 The disorder primarily affects males (male to female ratio, 9:1), and patients typically present between the ages of 20 and 50 years. There have been only a few reports of HES in children.3
References:
- Chusid MJ, Dale DC, West BC, Wolff SM. The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine (Baltimore). 1975;54:1-27.
- Karnak D, Kayacan O, Beder S, Delibalta M. Hypereosinophilic syndrome with pulmonary and cardiac involvement in a patient with asthma. CMAJ. 2003;168:172-175.
- Weller PF, Bubley GJ. The idiopathic hypereosinophilic syndrome. Blood. 1994;83:2759-2779.
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