Title: FIP1L1-PDGFR alpha, a therapeutic target for the treatment of chronic eosinophilic leukemia
Authors: Cools, Jan # ×
Issue Date: Aug-2005
Series Title: Verhandelingen - Koninklijke Academie voor Geneeskunde van België. vol:67 issue:3 pages:169-76
Abstract: The identification of the FIP1L1-PDGFRA fusion gene provides a molecular explanation for the pathogenesis of approximately half of the patients with the hypereosinophilic syndrome (HES). A diagnostic test to identify FIP1L1-PDGFRA positive HES cases (subsequently reclassified as chronic eosinophilic leukemia, CEL) is now available. FIP1L1-PDGFR alpha is a novel therapeutic target of the kinase inhibitor imatinib (Glivec, Novartis), which provides the basis for the treatment of these patients with this drug. FIP1L1-PDGFRA positive CEL patients respond very well to imatinib therapy, some of which are remarkable responses with normalization of the blood counts within 2 weeks after start of the therapy. Imatinib is well tolerated with minimal side effects, and most CEL patients respond to low doses of imatinib (100 mg/day), being important for lowering both the cost of therapy and drug related toxicity. All imatinib treated FIP1L1-PDGFRA positive CEL patients achieve hematological and cytogenetic remission, and the majority of patients also achieve a molecular remission with the fusion gene no longer detectable in blood, even by the most sensitive PCR techniques.
ISSN: 0302-6469
Publication status: published
KU Leuven publication type: AT
Appears in Collections:Molecular Genetics Section (-)
Department of Human Genetics - miscellaneous
× corresponding author
# (joint) last author

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