Idiopathic Hypereosinophilic Syndrome
|
0.600 |
Biomarker
|
disease |
GENOMICS_ENGLAND |
A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome.
|
12660384 |
2003 |
Idiopathic Hypereosinophilic Syndrome
|
0.600 |
Biomarker
|
disease |
BEFREE |
Recently, a novel tyrosine kinase that is generated from fusion of the Fip1-like 1 (FIP1L1) and PDGFR alpha (PDGFRA) genes has been identified as a therapeutic target for imatinib mesylate in hypereosinophilic syndrome (HES).
|
12842979 |
2003 |
Idiopathic Hypereosinophilic Syndrome
|
0.600 |
Biomarker
|
disease |
BEFREE |
The discovery of the FIP1L1-PDGFRA fusion gene in the hypereosinophilic syndrome is an example of the power of clinical translational research and identifies interstitial chromosomal deletion as a novel mechanism to generate oncogenic tyrosine kinase fusion genes.
|
14676627 |
2004 |
Idiopathic Hypereosinophilic Syndrome
|
0.600 |
Biomarker
|
disease |
BEFREE |
Eight had FIP1L1-PDGFRA (+) CEL, three had FIP1L1-PDGFRA (-) CEL and six had IHES.
|
14973504 |
2004 |
Idiopathic Hypereosinophilic Syndrome
|
0.600 |
GeneticVariation
|
disease |
BEFREE |
A pathogenetic mutation, FIP1L1-PDGFRA, that results from an interstitial chromosome 4q12 deletion, leads to a constitutive activation of the platelet-derived growth factor receptor-alpha (PDGFRA) tyrosine kinase as well as a disease phenotype that mimics both the hypereosinophilic syndrome (HES) and systemic mast cell disease associated with eosinophilia (SMCD-eos).
|
15036941 |
2004 |
Idiopathic Hypereosinophilic Syndrome
|
0.600 |
Biomarker
|
disease |
BEFREE |
Fusion of the Fip1-like 1 gene (FIP1L1) and the platelet-derived growth factor receptor alpha gene (PDGFRA) was discovered in the majority of patients with imatinib-sensitive HES, and all patients with the fusion responded to imatinib.
|
15175999 |
2004 |
Idiopathic Hypereosinophilic Syndrome
|
0.600 |
Biomarker
|
disease |
BEFREE |
Further investigation of the nature of FIP1L1-PDGFRA affected cells will improve the classification of HES.
|
15772698 |
2005 |
Idiopathic Hypereosinophilic Syndrome
|
0.600 |
Biomarker
|
disease |
BEFREE |
The hypereosinophilic syndrome: fluorescence in situ hybridization detects the del(4)(q12)-FIP1L1/PDGFRA but not genomic rearrangements of other tyrosine kinases.
|
15921374 |
2005 |
Idiopathic Hypereosinophilic Syndrome
|
0.600 |
Biomarker
|
disease |
BEFREE |
Discovery of the cryptic FIP1L1-PDGFRA gene fusion in cytogenetically normal patients with systemic mast cell disease with eosinophilia or idiopathic HES has redefined these diseases as clonal eosinophilias.
|
15995322 |
2005 |
Idiopathic Hypereosinophilic Syndrome
|
0.600 |
Biomarker
|
disease |
BEFREE |
Recent reports showed that a novel fusion tyrosine kinase, Fip1-like1 (FIP1L1) platelet-derived growth factor receptor alpha (PDGFRalpha), is found in idiopathic hypereosinophilic syndrome.
|
16343267 |
2006 |
Idiopathic Hypereosinophilic Syndrome
|
0.600 |
GeneticVariation
|
disease |
BEFREE |
Idiopathic hypereosinophilic syndrome (HES) in children is a very rare disorder; certain clinical differences with adult HES have been described, with no pediatric case with the imatinib-responsive FIP1L1-PDGFRA fusion gene reported to date.
|
16344672 |
2005 |
Idiopathic Hypereosinophilic Syndrome
|
0.600 |
Biomarker
|
disease |
BEFREE |
Multilineage involvement of the fusion gene in patients with FIP1L1/PDGFRA-positive hypereosinophilic syndrome.
|
16409293 |
2006 |
Idiopathic Hypereosinophilic Syndrome
|
0.600 |
AlteredExpression
|
disease |
BEFREE |
This study demonstrates the utility of screening for PDGFRA kinase domain overexpression in patients with IHES and has identified a third PDGFRA fusion partner in chronic myeloproliferative disorders.
|
16498388 |
2006 |
Idiopathic Hypereosinophilic Syndrome
|
0.600 |
GeneticVariation
|
disease |
BEFREE |
We tested the in vitro efficacy of imatinib and AMN107 in the EOL-1 cell line and in cells from a patient with HES harboring the FIP1L1-PDGFR-alpha fusion kinase.
|
16682077 |
2006 |
Idiopathic Hypereosinophilic Syndrome
|
0.600 |
Biomarker
|
disease |
CTD_human |
Hypereosinophilic syndrome (HES) has recently been recognized as a clonal leukemic lesion, which is due to a specific oncogenic event that generates hyperactive platelet-derived growth factor receptor-alpha-derived tyrosine kinase fusion proteins.
|
16778211 |
2006 |
Idiopathic Hypereosinophilic Syndrome
|
0.600 |
Biomarker
|
disease |
BEFREE |
Platelet-derived growth factor receptor-alpha-associated hypereosinophilic syndrome and lymphomatoid papulosis.
|
16965435 |
2006 |
Idiopathic Hypereosinophilic Syndrome
|
0.600 |
GeneticVariation
|
disease |
BEFREE |
Molecular analysis in peripheral blood did not reveal any mutation in the Fip1-like-platelet-derived growth factor receptor alpha chain (FIP1L1-PDGFRA) gene which was recently reported to be mutated in IHES.
|
16996710 |
2007 |
Idiopathic Hypereosinophilic Syndrome
|
0.600 |
GeneticVariation
|
disease |
BEFREE |
We screened seven Tunisian patients fulfilling the WHO criteria of HES for the presence of the FIP1L1-PDGFRA fusion gene using nested reverse transcription polymerase chain reaction on peripheral blood samples.
|
17137731 |
2007 |
Idiopathic Hypereosinophilic Syndrome
|
0.600 |
Biomarker
|
disease |
BEFREE |
Taken together, our data and previous reports suggest that FIP1L1-PDGFRA - positive HES is a distinct clinical entity with myeloproliferative features and showing a poor response to corticosteroid treatment.
|
17261495 |
2007 |
Idiopathic Hypereosinophilic Syndrome
|
0.600 |
Biomarker
|
disease |
BEFREE |
A 35-year-old man with FIP1L1-PDGFRA positive hypereosinophilic syndrome and cardiac involvement, was treated with imatinib 100 mg daily.
|
17544504 |
2008 |
Idiopathic Hypereosinophilic Syndrome
|
0.600 |
GeneticVariation
|
disease |
BEFREE |
The fusion protein between the platelet-derived growth factor receptor alpha (PDGFRalpha, P) gene and the Fip1-like1 (FIP1L1, F) may be identified in 14 to 60% of HES and it indicates a clonal hypereosinophilic syndrome called F/P-positive CEL.
|
17549478 |
2008 |
Idiopathic Hypereosinophilic Syndrome
|
0.600 |
Biomarker
|
disease |
BEFREE |
We consider the coadministration of corticosteroids and hydroxyurea to be an effective combination for the treatment of FIP1L1-PDGFRA-negative HES.
|
18004179 |
2007 |
Idiopathic Hypereosinophilic Syndrome
|
0.600 |
GeneticVariation
|
disease |
BEFREE |
Several reports of successful empirical treatment of idiopathic hypereosinophilic syndrome with imatinib led to the recent identification of the FIP1L1-PDGFRA fusion gene rearrangement, which characterizes a distinctive group of chronic eosinophilic leukemias.
|
18028420 |
2008 |
Idiopathic Hypereosinophilic Syndrome
|
0.600 |
Biomarker
|
disease |
BEFREE |
Interstitial deletion involving chromosome 4q12 generates the novel tyrosine kinase fusion protein encoded by FIP1L1-PDGFRA, which is present in many patients previously labelled as having hypereosinophilic syndrome, initially reported in 2003.
|
18256119 |
2008 |
Idiopathic Hypereosinophilic Syndrome
|
0.600 |
Biomarker
|
disease |
BEFREE |
Our study shows that treatment with mepolizumab, an agent designed to target eosinophils, can result in corticosteroid-sparing for patients negative for FIP1L1-PDGFRA who have the hypereosinophilic syndrome.
|
18344568 |
2008 |