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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.
The idiopathic hypereosinophilic syndrome is a disorder characterized by hypereosiophilia that is associated with eosinophil-based tissue or organ damage. While almost any organ or tissue may be damaged, the lung, skin, heart, blood vessels, sinuses, kidneys, and brain are the most commonly affected. [7]
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 underlying cause(s) for the origination and expansion of the phenotypically and clonally aberrant T cells in lymphocyte-variant hypereosinophilia remains unclear. In all events, these aberrant T cells are not, at least initially, malignant although they do exhibit pathological behavior.
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 third stage is a fibrotic stage, i.e. Loeffler endocarditis, wherein scarring replaces damaged heart muscle tissue to cause a poorly contracting heart and/or heart valve disease. Recent publications commonly refer to Loeffler endocarditis as a historical term for the third stage of eosinophilic myocarditis. [5] [6] [7]
A definitive diagnosis of EoE is based on the presence of at least 15 eosinophils/HPF in the esophageal biopsies of patients with exclusion of other causes of eosinophilia in the esophagus including gastroesophageal reflux disease (GERD), achalasia hypereosinophilic syndrome, Crohn's disease, infections, pill esophagitis, or graft vs host ...
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.