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Depending on eosinophil target-organ infiltration, the clinical presentation of hypereosinophilic syndrome (HES) varies from patient to patient. [13] Individuals with myeloproliferative variant HES may be more likely to experience mucosal ulcerations involving the genitalia or airways, while patients with lymphocytic variant HES typically exhibit prominent skin symptoms such as urticarial ...
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 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 ...
These mutations are recognized by the World Health Association as causing distinct entities differing from idiopathic hypereosinophilia and the idiopathic hypereosinophilic syndrome. Presence of these clones may be associated with tissue injury but in any case suggests specific therapy be directed at reducing the size and suppressing the growth ...
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]
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 ...
Glaxo's (GSK) Nucala becomes the first and only targeted biologic treatment to be approved for hypereosinophilic syndrome (HES), a rare disease caused by eosinophilic inflammation.
It can arise de novo or may develop in patients having the chronic form of a hypereosinophilic syndrome. Patients with acute eosinophilic leukemia have a propensity for developing bronchospasm as well as symptoms of the acute coronary syndrome and/or heart failure due to eosinophilic myocarditis and eosinophil-based endomyocardial fibrosis.