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BIOMARKER:

PDGFRA rearrangement

i
Other names: Platelet Derived Growth Factor Receptor Alpha, Platelet-Derived Growth Factor Receptor Alpha Polypeptide, Alpha-Type Platelet-Derived Growth Factor Receptor, CD140 Antigen-Like Family Member A, CD140a Antigen, PDGF-R-Alpha, PDGFR-2, PDGFR2, Alpha Platelet-Derived Growth Factor Receptor, Platelet-Derived Growth Factor Alpha Receptor, PDGFR-Alpha, RHEPDGFRA, CD140A
Entrez ID:
21d
Imatinib Treatment in Primary Acute Basophilic Leukemia with FIP1L1-PDGFRα Rearrangement: A Case Report. (PubMed, Acta Haematol)
Meanwhile, the FIP1L1::PDGFRA fusion gene detection results strongly support the use of molecular diagnosis in ABL. In addition, the patient successfully achieved a complete response to treatment and was followed for a year and a half without relapse, thus offering valuable insights for treatment strategies in similar cases.
Journal
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PDGFRA (Platelet Derived Growth Factor Receptor Alpha) • FIP1L1 (Factor Interacting With PAPOLA And CPSF1)
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FIP1L1-PDGFRA fusion • PDGFRA rearrangement
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imatinib
4ms
Case Report: Pathologic complete response in a patient with simultaneous diagnoses of resectable NSCLC and myeloid neoplasm with PDGFRA rearrangement treated with concurrent neoadjuvant chemoimmunotherapy and imatinib: translating clinical trial data to real-world practice. (PubMed, Front Oncol)
This case describes a 57-year-old man with simultaneous diagnoses of resectable stage IIIA NSCLC and PDGFRA-rearranged myeloid neoplasm who received neoadjuvant chemotherapy and nivolumab in combination with imatinib prior to definitive resection. Our report highlights the decision-making involved in pursuing combined systemic therapy of the patient's multiple malignancies and in navigating barriers related to tissue availability for biomarker testing. Approaches to neoadjuvant immunotherapy in early-stage NSCLC can be successful but must remain adaptable to reliably manage complex patient presentations in real-world, non-clinical trial settings.
Journal • Real-world evidence • PD(L)-1 Biomarker • IO biomarker
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PDGFRA (Platelet Derived Growth Factor Receptor Alpha)
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PDGFRA rearrangement
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Opdivo (nivolumab) • imatinib
7ms
Loeffler endocarditis revealing chronic eosinophilic leukaemia with FIP1L1-PDGFRA rearrangement: a case report. (PubMed, Eur Heart J Case Rep)
This case highlights that Loeffler endocarditis can present as the primary and sole manifestation of chronic eosinophilic leukaemia. Effective collaboration between cardiologists and internists is crucial for timely diagnosis and comprehensive management, ultimately resulting in enhanced outcomes.
Journal
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PDGFRA (Platelet Derived Growth Factor Receptor Alpha) • FIP1L1 (Factor Interacting With PAPOLA And CPSF1)
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FIP1L1-PDGFRA fusion • PDGFRA rearrangement
8ms
Nosologic reappraisal of the recently proposed calcified chondroid mesenchymal neoplasm concept in a series of 20 cases. (PubMed, Mod Pathol)
Using "CCMN" as an entity would entail mixed repercussions in diagnostic practice, and how and whether it should be used in diagnosis requires deliberate discussion. The concept might bring some benefits, but it may cause more confusion because it would substantially restructure the conventional framework by drawing a boundary within the chondroma and conflating the chondroma with chondroid tenosynovial giant cell tumor.
Journal
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FGFR2 (Fibroblast growth factor receptor 2) • PDGFRA (Platelet Derived Growth Factor Receptor Alpha) • MERTK (MER Proto-Oncogene, Tyrosine Kinase)
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FGFR2 fusion • PDGFRA rearrangement
9ms
FIP1L1::PDGFRA Fusion in a Pediatric Patient Presenting With B-Cell Lymphoblastic Leukemia. (PubMed, Genes Chromosomes Cancer)
Identification of FIP1L1::PDGFRA rearrangements is important as they enable treatment with a tyrosine kinase inhibitor, which has significantly improved the overall prognosis for PDGFRA-rearranged neoplasms. Prospective studies assessing imatinib dosage, duration, and long-term safety are warranted in this cohort.
Journal
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PDGFRA (Platelet Derived Growth Factor Receptor Alpha) • FIP1L1 (Factor Interacting With PAPOLA And CPSF1)
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FIP1L1-PDGFRA fusion • PDGFRA rearrangement
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imatinib
11ms
NCI-2018-03465: Azacitidine, Venetoclax, and Pevonedistat in Treating Patients With Newly Diagnosed Acute Myeloid Leukemia (clinicaltrials.gov)
P1/2, N=40, Active, not recruiting, M.D. Anderson Cancer Center | Trial completion date: Jan 2025 --> Jan 2027 | Trial primary completion date: Jan 2025 --> Jan 2027
Trial completion date • Trial primary completion date
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ABL1 (ABL proto-oncogene 1) • BCR (BCR Activator Of RhoGEF And GTPase) • NPM1 (Nucleophosmin 1) • PDGFRA (Platelet Derived Growth Factor Receptor Alpha) • PDGFRB (Platelet Derived Growth Factor Receptor Beta) • CEBPA (CCAAT Enhancer Binding Protein Alpha)
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NPM1 mutation • CEBPA mutation • PDGFRA rearrangement
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Venclexta (venetoclax) • azacitidine • pevonedistat (MLN4924)
almost2years
NCI-2018-03465: Azacitidine, Venetoclax, and Pevonedistat in Treating Patients With Newly Diagnosed Acute Myeloid Leukemia (clinicaltrials.gov)
P1/2, N=40, Active, not recruiting, M.D. Anderson Cancer Center | Trial completion date: Jan 2024 --> Jan 2025 | Trial primary completion date: Jan 2024 --> Jan 2025
Trial completion date • Trial primary completion date
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ABL1 (ABL proto-oncogene 1) • BCR (BCR Activator Of RhoGEF And GTPase) • NPM1 (Nucleophosmin 1) • PDGFRA (Platelet Derived Growth Factor Receptor Alpha) • PDGFRB (Platelet Derived Growth Factor Receptor Beta) • CEBPA (CCAAT Enhancer Binding Protein Alpha)
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NPM1 mutation • CEBPA mutation • PDGFRA rearrangement
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Venclexta (venetoclax) • azacitidine • pevonedistat (MLN4924)
2years
Retrospective Single Center Descriptive Analysis of a Cohort with Hypereosinophilia Evaluated in the Last Decade (ASH 2023)
HE requires a wide diagnostic evaluation with a multidisciplinary approach and the development of working groups in order to avoid these inconclusive diagnosis (12% in our series) and the missing diagnostic tests. Our study suggests the association between elevated LDH, cobalamins and tryptase and clonal cause of HE, as well as a higher rate of fibrosis, medullary dysplasia and altered karyotypes in these patients, indicating the need to include these evaluations in the diagnostic process. The group of PDGFRα patients had good prognosis with imatinib, but one patient presented clonal evolution and blastic transformation, showing the clinical challenge of these entities.
Retrospective data
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PDGFRA (Platelet Derived Growth Factor Receptor Alpha) • JAK2 (Janus kinase 2) • ETV6 (ETS Variant Transcription Factor 6)
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LDH elevation • BCR-JAK2 fusion • JAK2 fusion • PDGFRA rearrangement
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imatinib
over2years
Lymphomatoid papulosis associated with myeloid neoplasm with eosinophilia and FIP1L1::PDGFRA rearrangement: Successful imatinib treatment in two cases. (PubMed, J Dermatol)
Among eosinophilic disorders, the subtype identified as myeloid/lymphoid neoplasm with eosinophilia and tyrosine kinase gene fusions has aroused particular interest due to its excellent response to tyrosine kinase inhibitors, including imatinib. Here, we described the case of two 33-year-old men presenting with LyP and myeloid neoplasm with eosinophilia and FIP1L1::PDGFRA rearrangement who achieved complete clinical and molecular remission of both conditions a few months after starting imatinib.
Journal
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PDGFRA (Platelet Derived Growth Factor Receptor Alpha) • TNFRSF8 (TNF Receptor Superfamily Member 8) • FIP1L1 (Factor Interacting With PAPOLA And CPSF1)
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PDGFRA rearrangement
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imatinib