Journal article

Further evidence for the clonal nature of the idiopathic hypereosinophilic syndrome: complete haematological and cytogenetic remission induced by interferon-alpha in a case with a unique chromosomal abnormality

A 49-year-old man with the idiopathic hypereosinophilic syndrome (HES) and a unique chromosomal abnormality 46,XY,t(5;9)(q32;q33) is reported. Complete cytogenetic remission was induced by interferon alpha-2b (IFN-alpha). The beneficial action of IFN-alpha in different stem-cell disorders such as CML, HES, multiple myeloma and solid tumours such as hypernephroma or malignant melanoma suggests a common regulatory effect possibly by immunomodulation or other (immune-mediated) mechanisms, but the exact pathophysiological mechanisms remain hypothetic and unresolved. Since it has been known for some years that the genes encoding for GM-CSF, IL-3 and IL-5 reside on the long arm of chromosome 5, it could be possible that the chromosomal translocation in our patient resulted in excess production of these cytokines, hence causing the hypereosinophilia. This case report and the results obtained from the literature review support the growing body of evidence that IFN-alpha has a major place in the long-term treatment of HES, especially in those cases resistant to conventional treatment, with cytogenetic abnormalities, or presenting as a myeloproliferative variant of HES. In those cases IFN-alpha results in lower morbidity, lower mortality and long-term survival.

    Keywords: 99210-65-8 (Interferon Alfa-2b)



    Copyright 2003 U.S. National Library of Medicine

    Department of Haematology, UZ Gasthuisberg, Leuven, Belgium



    Journal; Article; (JOURNAL ARTICLE); General Review; (REVIEW); (REVIEW OF REPORTED CASES)



    Case Report; Human; Male

    Chromosomes, Human, Pair 5; Chromosomes, Human, Pair 9; Hematopoietic Stem Cells: PA, pathology; Hypereosinophilic Syndrome: BL, blood; *Hypereosinophilic Syndrome: GE, genetics; *Hypereosinophilic Syndrome: TH, therapy; *Interferon Alfa-2b: TU, therapeutic use; Middle Age; *Translocation (Genetics)


    Record created on 2011-02-01, modified on 2016-08-09


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