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.


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

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References in periodicals archive ?
A final diagnosis of idiopathic hypereosinophilic syndrome was made.
Kantarjian, "Response of idiopathic hypereosinophilic syndrome to treatment with imatinib mesylate," Leukemia Research, vol.
Gotlib et al., "A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome," New England Journal of Medicine, vol.
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).
Molecular characterization of the idiopathic hypereosinophilic syndrome (HES) in 35 French patients with normal conventional cytogenetics.
Clonal Th2 lymphocytes in patients with the idiopathic hypereosinophilic syndrome. Br J Haematol.
Idiopathic hypereosinophilic syndrome (HES) is a disease characterized by persistent hypereosinophilia (Greater than 1.5x109/L) for more than 6 months in the absence of other causes of reactive eosinophilia.
The diagnosis of idiopathic hypereosinophilic syndrome, therefore, is one of exclusion.
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.
In some of these it is believed that transforming growth factor-alpha (TGF-alpha) production, which is regulated by interleukin 3 (IL-3), IL-5, and granulocyte-macrophage colony-stimulating factor (GM-CSF) is unbalanced.(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.
Finally, chronic eosinophilic leukemia not otherwise specified, the lymphocytic and idiopathic hypereosinophilic syndromes, and paraneoplastic eosinophilia from occult solid organ tumors should only be considered when other etiologies have been excluded.

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