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.


Published in:
British Journal of Haematology, 92, 1, 176-183
Year:
1996
Keywords:
Note:
MEDLINE
Copyright 2003 U.S. National Library of Medicine
Department of Haematology, UZ Gasthuisberg, Leuven, Belgium
55
0372544
Journal; Article; (JOURNAL ARTICLE); General Review; (REVIEW); (REVIEW OF REPORTED CASES)
English
96136056
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)
Laboratories:




 Record created 2011-02-01, last modified 2018-01-28


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