BCR/ABL1-like acute lymphoblastic leukemia: How to diagnose and treat?

Cancer. 2019 Jan 15;125(2):194-204. doi: 10.1002/cncr.31848. Epub 2018 Dec 18.

Abstract

BCR/ABL1-like acute lymphoblastic leukemia (ALL) accounts for 15% to 30% of B-lineage ALL, with a peak of incidence occurring in adolescence. This subgroup of patients is characterized by a peculiar transcriptional profile that resembles that of true BCR/ABL1-positive cases, and have a heterogeneous genetic background and a poor outcome. Next-generation sequencing studies have demonstrated that the majority of patients carry rearrangements of tyrosine kinases or cytokine receptors and mutations of janus kinase (JAK)/signal transducer and activator of transcription (STAT), thus opening the way to the possible use of targeted therapeutic approaches. However, several issues remain unresolved at both the diagnostic and therapeutic level, such as the definition of a standardized method to identify BCR/ABL1-like ALL and the design of ad hoc clinical trials examining tyrosine kinase inhibitors or other tailored treatments. These aspects are discussed in this review.

Keywords: BCR/ABL1-like; Ph-like; acute lymphoblastic leukemia (ALL); diagnosis; prognosis; tyrosine kinase inhibitors (TKIs).

Publication types

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

MeSH terms

  • Animals
  • Female
  • Fusion Proteins, bcr-abl / genetics*
  • High-Throughput Nucleotide Sequencing
  • Humans
  • Janus Kinases / antagonists & inhibitors
  • Janus Kinases / genetics
  • Janus Kinases / metabolism
  • Male
  • Mutation
  • Peptide Fragments / therapeutic use
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / diagnosis*
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / drug therapy*
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / mortality

Substances

  • Peptide Fragments
  • protein kinase inhibitor peptide (5-24)
  • Fusion Proteins, bcr-abl
  • Janus Kinases