[Efficacy of imatinib in FIP1L1-PDGFRA positive hypereosinophilic syndrome].
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.
Menif
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S
Laboratoire d'hématologie moléculaire et cellulaire, institut Pasteur, Tunis, Tunisia. samia.menif@rns.tn
Ben Romdhane
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N
Hafsia
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R
D000328:Adult; D001549:Benzamides; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000188:drug therapy* / Q000235:genetics; D000068877:Imatinib Mesylate; D008297:Male; D008875:Middle Aged; D015514:Oncogene Proteins, Fusion / Q000187:drug effects*; D010879:Piperazines / Q000627:therapeutic use*; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use*; D011743:Pyrimidines / Q000627:therapeutic use*; D020796:Receptor, Platelet-Derived Growth Factor alpha / Q000187:drug effects*; D016896:Treatment Outcome; D039221:mRNA Cleavage and Polyadenylation Factors / Q000187:drug effects*
D001549:Benzamides; D015514:Oncogene Proteins, Fusion; D010879:Piperazines; D047428:Protein Kinase Inhibitors; D011743:Pyrimidines; D039221:mRNA Cleavage and Polyadenylation Factors; D000068877:Imatinib Mesylate; C479819:FIP1L1-PDGFRA fusion protein, human; D020796:Receptor, Platelet-Derived Growth Factor alpha