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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]
In most but not all instances, the resulting malignancies are associated with increases in blood, bone marrow, and/or tissue eosinophil levels as well as one or more of the signs, symptoms, tissue injuries, and organ dysfunctions (e.g. eosinophilic myocarditis) associated with the hypereosinophilic syndrome. The World Health Organization in ...
The hypereosinophilic syndrome is a sustained elevation in this count above 1.5 × 10 9 /L (i.e. 1,500/μL) that is also associated with evidence of eosinophil-based tissue injury. Eosinophils usually account for less than 7% of the circulating leukocytes. [ 1 ]
Idiopathic hypereosinophilic syndrome and lymphocyte-variant hypereosinophilia: corticosteroids; for individuals with these hypereosinophilias that are refractory to or breakthrough corticosteroid therapy and individuals requiring corticosteroid-sparing therapy, recommended alternative drug therapies include hydroxyurea, Pegylated interferon-α ...
The age-adjusted incidence of hypereosinophilic syndrome/chronic eosinophilic leukemia reported by the International Classification of Diseases for Oncology (Version 3) is ~0.036 per 100,000 with the mean frequency of FIP1L1-PDGFRA gene fusions occurring in ~10% of patients with hypereosinophilia as detected in developed countries. The fused ...
Familial eosinophilia is a rare congenital disorder characterized by the presence of sustained elevations in blood eosinophil levels that reach ranges diagnostic of eosinophilia (i.e. 500–1500/microliter) or, far more commonly, hypereosinophilia (i.e. >1,500/microliter).
Hypotrichosis–acro-osteolysis–onychogryphosis–palmoplantar keratoderma–periodontitis syndrome; Hypotrichosis–lymphedema–telangiectasia syndrome; Hystrix-like ichthyosis–deafness syndrome
Idiopathic hypereosinphilic syndrome and lymphocyte-variant hypereosinophilia: corticosteroids; for individuals with these hypereosinophilias that are refractory to or break through corticosteroid therapy and individuals requiring corticosteroid-sparing therapy, recommended alternative drug therapies include hydroxyurea, Pegylated interferon-α ...