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Hypereosinophilic syndrome is a disease characterized by a persistently elevated eosinophil count (≥ 1500 eosinophils/mm³) in the blood for at least six months without any recognizable cause, with involvement of either the heart, nervous system, or bone marrow. [5]
Eosinophilia is a condition in which the eosinophil count in the peripheral blood exceeds 5 × 10 8 /L (500/μL). [1] Hypereosinophilia is an elevation in an individual's circulating blood eosinophil count above 1.5 × 10 9 /L (i.e. 1,500/μL).
Eosinophilic granulomatosis with polyangiitis consists of three stages, but not all patients develop all three stages or progress from one stage to the next in the same order; [7] whereas some patients may develop severe or life-threatening complications such as gastrointestinal involvement and heart disease, some patients are only mildly affected, e.g. with skin lesions and nasal polyps. [8]
This distinction is important because the eosinophil-based disorder is due to a particular set of underlying diseases and its preferred treatments differ from those for non-eosinophilic myocarditis. [1] [2] Eosinophilic myocarditis is often viewed as a disorder that has three progressive stages.
Criteria for the diagnosis include findings of: a) long term hypereosinophilia (i.e. eosinophil blood counts >1,500/microliter) plus physical findings and symptoms associated with the disease; b) bone marrow analysis showing abnormally high levels of eosinophils; c) elevated serum levels of Immunoglobulin E, other immunoglobulins, and CCL17; d ...
An IgE level greater than 2,000 IU/mL is often considered diagnostic. [17] However, patients younger than 6 months of age may have very low to non-detectable IgE levels. Eosinophilia is also a common finding with greater than 90% of patients having eosinophil elevations greater than two standard deviations above the normal mean. [18]
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