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BIOMARKER:

FIP1L1-PDGFRA rearrangement

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Other names: FIP1L1, Factor Interacting With PAPOLA And CPSF1, FIP1L1 Cleavage And Polyadenylation Specific Factor Subunit, Pre-MRNA 3'-End-Processing Factor FIP1, Rearranged In Hypereosinophilia, Factor Interacting With PAP, FIP1-Like 1 Protein, HFip1, FIP1, FIP1 Like 1 (S. Cerevisiae), FIP1 Like 1, RHE, 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 Antig
Entrez ID:
over1year
Lymphomatoid Papulosis Associated with Myeloid Neoplasm with Eosinophilia and FIP1L1-Pdgfra Rearrangement: Successful Imatinib Treatment in Two Cases (ASH 2022)
Due to recurrence of erythematous papules followed by necrotic evolution, methotrexate 10 mg once weekly was started with symptoms resolution. In some cases, it may be linked to the release of eosinophilopoietic cytokines, such as IL-5 and IL-3, by abnormal LyP T lymphocytes. In addition, FIP1L1-PDGFRA rearrangement has been identified in both eosinophils and clonal T cells in some patients, thus suggesting a common pathogenetic mechanism and the reason why imatinib is capable of inducing a clinical-molecular remission of both conditions.Consequently, search for FIP1L1-PDGFRA rearrangement should always be performed in LyP patients who present with HE at diagnosis or develop it during follow-up.
Clinical
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ABL1 (ABL proto-oncogene 1) • BCR (BCR Activator Of RhoGEF And GTPase) • FGFR1 (Fibroblast growth factor receptor 1) • PDGFRA (Platelet Derived Growth Factor Receptor Alpha) • JAK2 (Janus kinase 2) • CD8 (cluster of differentiation 8) • TNFRSF8 (TNF Receptor Superfamily Member 8) • PDGFRB (Platelet Derived Growth Factor Receptor Beta) • CD4 (CD4 Molecule) • FIP1L1 (Factor Interacting With PAPOLA And CPSF1) • CD5 (CD5 Molecule) • IRF4 (Interferon regulatory factor 4) • PCM1 (Pericentriolar Material 1) • CD7 (CD7 Molecule) • CD2 (CD2 Molecule) • IL5 (Interleukin 5)
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BCR-ABL1 fusion • PDGFRA mutation • JAK2 V617F • FIP1L1-PDGFRA rearrangement • PDGFRA rearrangement
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imatinib • methotrexate