Myeloid/Lymphoid Neoplasms with FGFR1 Rearrangement
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0.100 |
GeneticVariation
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disease |
BEFREE |
We report a rare case of t(8;9)(p11;q33) in a patient with 8p11 myeloproliferative syndrome (EMS) that was preceded by centrosomal protein 110kDa (CEP110; previously CEP1)/fibroblast growth factor receptor 1 (FGFR1) fusion transcript.
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18295660 |
2008 |
Myeloid/Lymphoid Neoplasms with FGFR1 Rearrangement
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0.100 |
Biomarker
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disease |
BEFREE |
We demonstrate that ZNF198-FGFR1 induces EMS-like disease in mice, with myeloproliferation and T lymphoma arising from common multipotential progenitors.
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15050920 |
2004 |
Myeloid/Lymphoid Neoplasms with FGFR1 Rearrangement
|
0.100 |
GeneticVariation
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disease |
BEFREE |
We conclude that 1) all mRNA fusions in EMS result in splicing to FGFR1 exon 9 but breakpoints in FOP are variable, 2) two-color FISH can identify patients with EMS, and 3) the t(8;17)(p11;q25), t(8;11)(p11;p15), t(8;12)(p11;q15), and ins(12;8)(p11;p11p21) are novel karyotypic changes that most likely involve FGFR1.
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11550283 |
2001 |
Myeloid/Lymphoid Neoplasms with FGFR1 Rearrangement
|
0.100 |
Biomarker
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disease |
BEFREE |
To identify the most promising drug for EMS, the activities and associated mechanism of three tyrosine kinase inhibitors (TKIs), TKI258, ponatinib and AZD4547, against TPR‑FGFR1 were tested by MTT assay, flow cytometry and western blot.
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28138694 |
2017 |
Myeloid/Lymphoid Neoplasms with FGFR1 Rearrangement
|
0.100 |
GeneticVariation
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disease |
BEFREE |
To date, four gene fusions associated with distinct translocations have been described in EMS: the t(8;13)(p11;q12), t(8;9)(p11;q33), t(6;8)(q27;p11) and t(8;22)(p11q22) fuse ZNF198, CEP110, FOP and BCR, respectively, to FGFR1.
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11919391 |
2002 |
Myeloid/Lymphoid Neoplasms with FGFR1 Rearrangement
|
0.100 |
Biomarker
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disease |
BEFREE |
Thus, our data extend the involvement of FGFR1 in EMS and lend support to the concept that there is a precise correlation between genotype and phenotype in this disease.
|
15609342 |
2005 |
Myeloid/Lymphoid Neoplasms with FGFR1 Rearrangement
|
0.100 |
Biomarker
|
disease |
BEFREE |
These data further support the central role of deregulated FGFR1 in the pathogenesis of EMS.
|
15034873 |
2004 |
Myeloid/Lymphoid Neoplasms with FGFR1 Rearrangement
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
The ZNF198- FGFR1 fusion gene arises as a result of the t(8;13)(p11;q12) in the 8p11 myeloproliferative syndrome.
|
10935490 |
1999 |
Myeloid/Lymphoid Neoplasms with FGFR1 Rearrangement
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
The t(8;17)(p11;q23) in the 8p11 myeloproliferative syndrome fuses MYO18A to FGFR1.
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15800673 |
2005 |
Myeloid/Lymphoid Neoplasms with FGFR1 Rearrangement
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
The t(8;13)(p11;q12) is the most common translocation associated with the 8p11 myeloproliferative syndrome and results in an identical mRNA fusion between ZNF198 at 13q12 and FGFR1 at 8p11 in all cases thus far reported.
|
9889006 |
1999 |
Myeloid/Lymphoid Neoplasms with FGFR1 Rearrangement
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
The 8p11 myeloproliferative syndrome (EMS), a rare myeloproliferative disease, generally progresses rapidly and is characterized by chromosomal translocations of the fibroblast growth factor receptor 1 (FGFR1) gene.
|
30116393 |
2018 |
Myeloid/Lymphoid Neoplasms with FGFR1 Rearrangement
|
0.100 |
AlteredExpression
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disease |
BEFREE |
The 8p11 myeloproliferative syndrome (EMS) is an aggressive chronic myeloproliferative disorder (MPD) that is caused by constitutive activation of fibroblast growth factor receptor 1.
|
25803811 |
2015 |
Myeloid/Lymphoid Neoplasms with FGFR1 Rearrangement
|
0.100 |
GeneticVariation
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disease |
BEFREE |
The 8p11 myeloproliferative syndrome is an aggressive neoplasm associated with chromosomal abnormalities involving rearrangement of the fibroblast growth factor receptor 1 (FGFR1) gene.
|
25037443 |
2014 |
Myeloid/Lymphoid Neoplasms with FGFR1 Rearrangement
|
0.100 |
GeneticVariation
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disease |
BEFREE |
The 8p11 myeloproliferative syndrome is a rare atypical disorder defined by the presence of rearrangements between the fibroblast growth factor receptor 1 (FGFR1) and 1 of 13 partner genes described to date, including the BCR gene on chromosome 22.
|
23519513 |
2013 |
Myeloid/Lymphoid Neoplasms with FGFR1 Rearrangement
|
0.100 |
GeneticVariation
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disease |
BEFREE |
The 8p11 myeloproliferative syndrome is a rare hematologic malignancy derived from a pluripotent hematopoietic stem cell associated with rearrangements involving the fibroblast growth factor receptor 1 (FGFR1) gene located on chromosome 8p11.
|
19829149 |
2009 |
Myeloid/Lymphoid Neoplasms with FGFR1 Rearrangement
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
The 8p11 myeloproliferative syndrome (EMS) is associated with translocations that disrupt the FGFR1 gene.
|
16946300 |
2006 |
Myeloid/Lymphoid Neoplasms with FGFR1 Rearrangement
|
0.100 |
Biomarker
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disease |
BEFREE |
Primary cells in BCR/FGFR1-positive 8p11 myeloproliferative syndrome are sensitive to dovitinib, ponatinib, and dasatinib.
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28881484 |
2017 |
Myeloid/Lymphoid Neoplasms with FGFR1 Rearrangement
|
0.100 |
Biomarker
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disease |
BEFREE |
Ponatinib as targeted therapy for FGFR1 fusions associated with the 8p11 myeloproliferative syndrome.
|
22875613 |
2013 |
Myeloid/Lymphoid Neoplasms with FGFR1 Rearrangement
|
0.100 |
Biomarker
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disease |
BEFREE |
Our results further support a critical role of FGFR1 in the pathogenesis of EMS and may lead to more accurate diagnosis and potential targeted therapy.
|
22619110 |
2012 |
Myeloid/Lymphoid Neoplasms with FGFR1 Rearrangement
|
0.100 |
GeneticVariation
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disease |
BEFREE |
Of the 2 cases of EMS with t(1;8)(q25;p11.2) which were previously reported, FGFR1 rearrangement was not confirmed in 1 case.
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25227135 |
2015 |
Myeloid/Lymphoid Neoplasms with FGFR1 Rearrangement
|
0.100 |
GeneticVariation
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disease |
BEFREE |
It is important to recognize that neoplasms carrying the t(8;22)/BCR-FGFR1, although rare, can commonly with B lymphoblastic leukemia at the initial diagnosis, which could distract one from recognizing a possible underlying 8p11 myeloproliferative syndrome.
|
28551329 |
2017 |
Myeloid/Lymphoid Neoplasms with FGFR1 Rearrangement
|
0.100 |
GeneticVariation
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disease |
BEFREE |
In the present study, we have investigated a case of EMS harboring a t(8;22)(p11;q11)/BCR-FGFR1 rearrangement as well as a t(9;21)(q34;q22) at the time of AML transformation.
|
17394134 |
2007 |
Myeloid/Lymphoid Neoplasms with FGFR1 Rearrangement
|
0.100 |
Biomarker
|
disease |
BEFREE |
In the future, FGFR1 inhibitors might be specific and effective therapeutic targets for EMS.
|
29614500 |
2019 |
Myeloid/Lymphoid Neoplasms with FGFR1 Rearrangement
|
0.100 |
Biomarker
|
disease |
BEFREE |
In the future, FGFR1 inhibitor might be the specific and effective therapeutic target for EMS.
|
21214407 |
2011 |
Myeloid/Lymphoid Neoplasms with FGFR1 Rearrangement
|
0.100 |
Biomarker
|
disease |
BEFREE |
In the current World Health Organization classification, the 8p11 myeloproliferative syndrome is designated as "myeloid and lymphoid neoplasms with FGFR1 abnormalities."
|
20226962 |
2010 |