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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
Author(s) -
Malbrain Manu,
Van den Bergh Herman,
Zachée Pierre
Publication year - 1996
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1046/j.1365-2141.1996.00298.x
Subject(s) - hypereosinophilia , hypereosinophilic syndrome , alpha interferon , chromosomal translocation , immunology , cytogenetics , biology , pathophysiology , medicine , cancer research , chromosome , interferon , eosinophilia , gene , genetics
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‐α). The beneficial action of IFN‐α 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‐α 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‐α results in lower morbidity, lower mortality and long‐term survival.