RGD Reference Report - Response to imatinib mesylate in patients with hypereosinophilic syndrome. - Rat Genome Database

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Response to imatinib mesylate in patients with hypereosinophilic syndrome.

Authors: Arefi, M  Garcia, JL  Briz, MM  De Arriba, F  Rodriguez, JN  Martin-Nunez, G  Martinez, J  Lopez, J  Suarez, JG  Moreno, MJ  Merino, MA  Gutierrez, NC  Hernandez-Rivas, JM 
Citation: Arefi M, etal., Int J Hematol. 2012 Sep;96(3):320-6. doi: 10.1007/s12185-012-1141-7. Epub 2012 Jul 18.
RGD ID: 11075089
Pubmed: PMID:22806436   (View Abstract at PubMed)
DOI: DOI:10.1007/s12185-012-1141-7   (Journal Full-text)

Idiopathic hypereosinophilic syndrome (HES) is a rare disorder characterized by unexplained, persistent hypereosinophilia associated with multiple organ dysfunctions. The cause of HES is unknown and shows clinical heterogeneity. FIP1L1-PDGFRA fusion is a clonal marker for the diagnosis and treatment of HES. We prospectively studied 78 patients with chronic eosinophilia. In all cases, the most salient clinical and biological characteristics as well as the response to the therapy were analyzed. In addition, we performed conventional cytogenetics and fluorescent in situ hybridization (FISH) with three BACs covering the FIP1-like-1 (FIP1L1)/platelet-derived growth factor receptor-alpha gene (PDGFRA) fusion. Nineteen of 78 patients (24 %) presented criteria of HES. The majority of patients were male (18) with median age of 49 years (range 19-84 years). FIP1L1-PDGFRA fusion was found in eight patients. Patients with FIP1L1-PDGFRA fusion presented with more bone marrow eosinophils and peripheral blood eosinophilia as well as anemia, leukocytosis and thrombocytopenia. Using of low-dose imatinib mesylate (100 mg/day) a hematological and molecular remission in all patients displaying the FIP1L1-PDGFRA fusion gene was observed. Therefore, imatinib may be effective for use in the treatment of chronic eosinophilic leukemia, and patients should be treated before tissue damage.



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Manual Human Phenotype Annotations - RGD

Object SymbolSpeciesTermQualifierEvidenceWithNotesSourceOriginal Reference(s)
FIP1L1HumanChronic leukemia  IAGP DNA:gene fusionRGD 
PDGFRAHumanChronic leukemia  IAGP DNA:gene fusionRGD 
Objects Annotated

Genes (Rattus norvegicus)
Fip1l1  (factor interacting with PAPOLA and CPSF1)
Pdgfra  (platelet derived growth factor receptor alpha)

Genes (Mus musculus)
Fip1l1  (factor interacting with PAPOLA and CPSF1)
Pdgfra  (platelet derived growth factor receptor, alpha polypeptide)

Genes (Homo sapiens)
FIP1L1  (factor interacting with PAPOLA and CPSF1)
PDGFRA  (platelet derived growth factor receptor alpha)


Additional Information