RNA Sequencing Analysis for the Identification of a PCM1/PDGFRB Fusion Gene Responsive to Imatinib

Acta Haematol. 2019;142(2):92-97. doi: 10.1159/000497348. Epub 2019 May 14.

Abstract

The platelet-derived growth factor receptor β (PDGFRB) gene translocations lead to a spectrum of chronic myeloid neoplasms, frequently associated with eosinophilia. Clinical heterogeneity is associated with a molecular one. Here, we report a novel case of a patient harboring a t(5;8)(q33;p22) translocation, resulting in the PCM1/PDGFRB fusion. Conventional cytogenetics and RNA sequencing were performed to identify the chromosomes and the genes involved in the rearrangement, respectively. This study shows that the combination of different strategies is pivotal to fine-tune the diagnosis and the clinical management of the patient. After 1 year of treatment with imatinib, the patient achieves hematological and molecular remission. We present an attractive strategy to identify novel and/or cryptic fusions, which will be relevant for clinicians dealing with the diagnosis of the patients with myelodysplastic syndrome/myeloproliferative diseases with atypical manifestations.

Keywords: Fusion; Imatinib; Myeloid neoplasm; PCM1; PDGFRB; RNA sequencing; Translocation.

Publication types

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

MeSH terms

  • Autoantigens* / genetics
  • Autoantigens* / metabolism
  • Cell Cycle Proteins* / genetics
  • Cell Cycle Proteins* / metabolism
  • Chromosomes, Human, Pair 5 / genetics
  • Chromosomes, Human, Pair 5 / metabolism
  • Chromosomes, Human, Pair 8 / genetics
  • Chromosomes, Human, Pair 8 / metabolism
  • Humans
  • Imatinib Mesylate / administration & dosage*
  • Leukemia, Myelogenous, Chronic, BCR-ABL Positive* / drug therapy
  • Leukemia, Myelogenous, Chronic, BCR-ABL Positive* / genetics
  • Leukemia, Myelogenous, Chronic, BCR-ABL Positive* / metabolism
  • Leukemia, Myelogenous, Chronic, BCR-ABL Positive* / pathology
  • Male
  • Middle Aged
  • Oncogene Proteins, Fusion* / genetics
  • Oncogene Proteins, Fusion* / metabolism
  • Receptor, Platelet-Derived Growth Factor beta* / genetics
  • Receptor, Platelet-Derived Growth Factor beta* / metabolism
  • Sequence Analysis, RNA*
  • Translocation, Genetic

Substances

  • Autoantigens
  • Cell Cycle Proteins
  • Oncogene Proteins, Fusion
  • PCM1 protein, human
  • Imatinib Mesylate
  • PDGFRB protein, human
  • Receptor, Platelet-Derived Growth Factor beta