The Current Genomic and Molecular Landscape of Philadelphia-like Acute Lymphoblastic Leukemia

Int J Mol Sci. 2020 Mar 22;21(6):2193. doi: 10.3390/ijms21062193.

Abstract

Philadelphia (Ph)-like acute lymphoblastic leukemia (ALL) is a high-risk B-cell Acute Lymphoblastic Leukemia (B-ALL) characterized by a gene expression profile similar to Ph-positive B-ALL but lacking the BCR-ABL1 translocation. The molecular pathogenesis of Ph-like B-ALL is heterogenous and involves aberrant genomics, receptor overexpression, kinase fusions, and mutations leading to kinase signaling activation, leukemogenic cellular proliferation, and differentiation blockade. Testing for the Ph-like signature, once only a research technique, is now available to the clinical oncologist. The plethora of data pointing to poor outcomes for this ALL subset has triggered investigations into the role of targeted therapies, predominantly involving tyrosine kinase inhibitors that are showing promising results.

Keywords: CRLF2; PI3K/AKT/mTOR; acute lymphoblastic leukemia; targeted therapy.

Publication types

  • Review

MeSH terms

  • Animals
  • Fusion Proteins, bcr-abl / genetics
  • Gene Expression Regulation, Leukemic
  • Genomics
  • Humans
  • Ikaros Transcription Factor / genetics
  • PAX5 Transcription Factor / genetics
  • Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / diagnosis
  • Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / genetics
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / diagnosis
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics*
  • Receptors, Cytokine / genetics
  • Transcriptome*

Substances

  • CRLF2 protein, human
  • PAX5 Transcription Factor
  • PAX5 protein, human
  • Receptors, Cytokine
  • Ikaros Transcription Factor
  • Fusion Proteins, bcr-abl