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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]
Clonal hypereosinophilia, also termed primary hypereosinophilia or clonal eosinophilia, is a grouping of hematological disorders all of which are characterized by the development and growth of a pre-malignant or malignant population of eosinophils, a type of white blood cell that occupies the bone marrow, blood, and other tissues.
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 ]
Reslizumab, a newly developed antibody directed against interleukin 5 that has been successfully used to treat 4 patients with the hypereosinophilic syndrome, may also be of use for lymphocyte-variant eosinophilia. [4] [5] [10] [11] Patients suffering minimal or no disease complications have gone untreated. [4]
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 ...
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 ...
Signs and symptoms may include weight loss, fever, malaise, cough, skin and mucosal lesions, diarrhea, and peripheral neuropathy.Cardiac symptoms are also possible. [2]In cases associated with PDGFRB and FGFR1 mutations, splenomegaly is common.
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 ...