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BIOMARKER:

ETV6-PDGFRB fusion

i
Other names: ETV6, ETS Variant Transcription Factor 6, ETS Variant 6, Ets Variant Gene 6 (TEL Oncogene), ETS Translocation Variant 6, Transcription Factor ETV6, ETS-Related Protein Tel1, TEL, TEL1 Oncogene, TEL Oncogene, TEL/ABL, THC5, TEL1, Tel, PDGFRB, Platelet Derived Growth Factor Receptor Beta, Platelet-Derived Growth Factor Receptor, Beta Polypeptide, Beta-Type Platelet-Derived Growth Factor Receptor, Platelet-Derived Growth Factor Receptor Beta, Platelet-Derived Growth Factor Receptor 1, CD140 Antigen
Entrez ID:
12ms
A SINGLE CENTER EXPERIENCE OF DIAGNOSTICS AND TREATMENT OF RARE MYELOID/LYMPHOID NEOPLASMS WITH PDGFRB GENE REARRANGEMENT (EHA 2023)
A high sensitivity to targeted therapy with imatinib (IM) is expected... A long-term experience of rare PDGFRB –positive diseases observation reveals that FISH is an optimal method for detection of various PDGFRB gene rearrangements and it should be done in all pts with HES and previously excluded PDGFRA (including those with unchanged karyotype). The IM effectiveness in PDGFRB -positive neoplasms is ambiguous which may be due to the partner gene of PDGFRB. The choice of IM dose should be 400 mg QD.
Clinical
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PDGFRA (Platelet Derived Growth Factor Receptor Alpha) • PDGFRB (Platelet Derived Growth Factor Receptor Beta) • ETV6 (ETS Variant Transcription Factor 6)
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ETV6-PDGFRB fusion • PDGFRB fusion
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imatinib
over1year
Myeloid neoplasm with ETV6::ACSl6 fusion: landscape of molecular and clinical features. (PubMed, Hematology)
The treatments and outcomes varied greatly depending on the type of disease, although tyrosine kinase inhibitors (TKIs) were not effective. In contrast to neoplasms with ETV6::PDGFRB fusion, myeloid neoplasms with ETV6::ACSL6 fusion have unique characteristics.
Journal
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PDGFRB (Platelet Derived Growth Factor Receptor Beta) • ETV6 (ETS Variant Transcription Factor 6)
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ETV6-PDGFRB fusion • PDGFRB fusion