idiopathic hypereosinophilic syndrome

idiopathic hypereosinophilic syndrome

(hī″pĕ-rē″ŏ-sin″ŏ-fil′ik) [ hyper- + eosinophilic]
Multisystem injury and organ damage caused by excessive numbers of eosinophils in the body. The disease is one of the myelodysplastic disorders. Almost any organ can be affected, but most patients have bone marrow, cardiac, and central nervous system involvement.

Treatment

Corticosteroids are given. Additional therapies include immune-suppressing agents, (e.g., cyclosporine or interferon) or cancer chemotherapy.

References in periodicals archive ?
Protein C and S deficiency, antiphospolipid syndrome, myeloproliferative disorders, idiopathic hypereosinophilic syndrome, pheochromocytoma, and Takatsubo cardiomyopathy were reported to be related with thrombus formation in patients with a normal LV ejection fraction (8-10).
A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome.
Molecular characterization of the idiopathic hypereosinophilic syndrome (HES) in 35 French patients with normal conventional cytogenetics.
The eosinophilias, including the idiopathic hypereosinophilic syndrome.
Elevated serum tryptase levels identify a subset of patients with a myeloproliferative variant of idiopathic hypereosinophilic syndrome associated with tissue fibrosis, poor prognosis, and imatinib responsiveness.
The idiopathic hypereosinophilic syndrome was first defined by Chusid et al in 1975 as (1) persistent eosinophilia of 1.
Coverage includes techniques for the detection of BCR-ABL mutations and resistance to imatinib mesylate, detection of the FIP 1L1-PDGFRA fusion in idiopathic hypereosinophilic syndrome and chronic eosinophilic leukemia, classification of acute myeloid leukemia by DNA- oligonucleotide microarrays, detection of the V617F JAK2 mutation in myeloproliferative disorders, gene rearrangements, FLT3 mutations andWT-1 overexpression.
17) Eosinophilia may be observed in: (1) benign reactive eosinophilia, (2) eosinophilia of miscellaneous malignant disorders, and (3) sustained idiopathic eosinophilia, including idiopathic hypereosinophilic syndrome and eosinophilic leukemia.
Response of idiopathic hypereosinophilic syndrome to treatment with imatinib mesylate.

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