Targeted therapy and disease monitoring in CNTRL-FGFR1-driven leukaemia

Pediatr Blood Cancer. 2019 Oct;66(10):e27897. doi: 10.1002/pbc.27897. Epub 2019 Jun 28.

Abstract

We report two patients with leukaemia driven by the rare CNTRL-FGFR1 fusion oncogene. This fusion arises from a t(8;9)(p12;q33) translocation, and is a rare driver of biphenotypic leukaemia in children. We used RNA sequencing to report novel features of expressed CNTRL-FGFR1, including CNTRL-FGFR1 fusion alternative splicing. From this knowledge, we designed and tested a Droplet Digital PCR assay that detects CNTRL-FGFR1 expression to approximately one cell in 100 000 using fusion breakpoint-specific primers and probes. We also utilised cell-line models to show that effective tyrosine kinase inhibitors, which may be included in treatment regimens for this disease, are only those that block FGFR1 phosphorylation.

Keywords: fusion gene; leukaemia; minimal residual disease monitoring; targeted therapies; tyrosine kinase.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Cell Cycle Proteins / genetics*
  • Child
  • Humans
  • Infant
  • Leukemia / genetics*
  • Leukemia / therapy*
  • Male
  • Molecular Targeted Therapy / methods*
  • Oncogene Fusion
  • Oncogene Proteins, Fusion / genetics
  • Polymerase Chain Reaction / methods
  • Protein Kinase Inhibitors / therapeutic use
  • Receptor, Fibroblast Growth Factor, Type 1 / genetics*

Substances

  • CNTRL protein, human
  • Cell Cycle Proteins
  • Oncogene Proteins, Fusion
  • Protein Kinase Inhibitors
  • FGFR1 protein, human
  • Receptor, Fibroblast Growth Factor, Type 1