Cell clonality in hypereosinophilic syndrome : what pathogenetic role?

Idiopathic hypereosinophilic syndrome (HES) is a heterogeneous disorder, including either a myeloproliferative or a lymphoproliferative variant (l-HES). In l-HES, T-lymphocytes could be involved in the pathogenesis through several cytokines, including IL5. We assayed both TCR Beta- and delta-rearran...

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Bibliographic Details
Published in:Clinical and experimental rheumatology 2007, Vol.25 (1), p.17-22
Main Authors: GALIMBERTI, S, CIABATTI, E, OTTIMO, F, ROSSI, A, TROMBI, L, CARULLI, G, CERVETTI, G, MATTII, L, BIANCHI, G, PETRINI, M
Format: Article
Language:English
Subjects:
Adult
Aged
Benzamides
Biological and medical sciences
Cytokines - analysis
Cytokines - genetics
Eosinophils - classification
Eosinophils - drug effects
Eosinophils - pathology
Female
Hematologic and hematopoietic diseases
Humans
Hypereosinophilic Syndrome - genetics
Hypereosinophilic Syndrome - immunology
Hypereosinophilic Syndrome - physiopathology
Imatinib Mesylate
Immunologic Factors - pharmacology
Interferon-alpha - pharmacology
Male
Medical sciences
Middle Aged
mRNA Cleavage and Polyadenylation Factors - blood
Oncogene Proteins, Fusion - blood
Other diseases. Hematologic involvement in other diseases
Piperazines - pharmacology
Protein Kinase Inhibitors - pharmacology
Pyrimidines - pharmacology
Receptor, Platelet-Derived Growth Factor alpha - blood
Retrospective Studies
T-Lymphocytes - metabolism
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Summary:Idiopathic hypereosinophilic syndrome (HES) is a heterogeneous disorder, including either a myeloproliferative or a lymphoproliferative variant (l-HES). In l-HES, T-lymphocytes could be involved in the pathogenesis through several cytokines, including IL5. We assayed both TCR Beta- and delta-rearrangements by fluorescent PCR, characterizing 14 patients affected by HES. Lyn activation (a src-kinase involved in the IL5 pathway) was also tested in 6 cases. FIP1L1-PDGFRa was detected in 4 cases (28.6%); a clonal TCR was found in 10 cases (71.4%), including cases FIP1L1-PDGFRalpha-positive; four cases did not show any molecular marker. In this series, levels of IL5, IL4, IL2 and gammaIFN were measured, without any significant difference among different subgroups. All pathological samples tested did not show Lyn activation. Immunophenotype was also characterized: only one case showed an atypical CD3-/CD4+ population in the bone marrow. This study would suggest that a real distinction between m- and l-HES is not wholly convincing and that clonal T-cell expansion could not be the "primum movens" but an epiphenomenon in HES.
ISSN:0392-856X
1593-098X