[Efficacy of imatinib in FIP1L1-PDGFRA positive hypereosinophilic syndrome]. / Efficacité de l'imatinib dans les syndromes hyperéosinophiliques associés au transcrit FIP1L1-PDGFRA.
Rev Med Interne
; 29(1): 65-7, 2008 Jan.
Article
in Fr
| MEDLINE
| ID: mdl-18036706
ABSTRACT
Hypereosinophilic syndromes (HES) are a heterogeneous group of disorders characterized by marked peripheral blood and tissue eosinophilia resulting in organ damage. Recent advances in molecular biology have led to the identification of a FIP1L1-PDGFRA fusion gene as a recurrent abnormality in some patients with HES. This fusion gene results from a cryptic 4q12 interstitial deletion involving an 800 kb region. Recent reports indicate that this subtype of HES is imatinib responsive with rapid and complete haematological remissions. Here we report two patients successfully treated with imatinib.
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Collection:
01-internacional
Database:
MEDLINE
Main subject:
Piperazines
/
Pyrimidines
/
Oncogene Proteins, Fusion
/
Hypereosinophilic Syndrome
/
Receptor, Platelet-Derived Growth Factor alpha
/
MRNA Cleavage and Polyadenylation Factors
/
Protein Kinase Inhibitors
Type of study:
Prognostic_studies
Limits:
Adult
/
Humans
/
Male
/
Middle aged
Language:
Fr
Journal:
Rev Med Interne
Year:
2008
Document type:
Article
Affiliation country: