Philadelphia-like acute lymphoblastic leukemia: diagnostic dilemma and management perspectives

Exp Hematol. 2018 Nov:67:1-9. doi: 10.1016/j.exphem.2018.07.007. Epub 2018 Jul 31.

Abstract

Acute lymphoblastic leukemia (ALL) is an aggressive hematologic malignancy characterized by suboptimal outcomes in the adult age group. Recently, a new subtype called Philadelphia (Ph)-like ALL has been described. This subgroup is characterized by high cytokine receptor and tyrosine kinase signaling expression, resulting in kinase activation through stimulation of two main pathways, the ABL and JAK/STAT pathways. The diagnostic method or approach for Ph-like ALL is still not standardized and efforts are ongoing to identify an easy and applicable diagnostic method. Accurate and standard testing approaches are much needed and this will facilitate better understanding of this subgroup, including better estimation of the prevalence and incidence in different age groups and the clinical outcomes of such new entity. Here, we review the currently available diagnostic tools, activated pathways, and different therapeutic approaches used to target this subgroup.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Adult
  • Age Distribution
  • Aged
  • Aged, 80 and over
  • Antineoplastic Agents / pharmacology
  • Antineoplastic Agents / therapeutic use
  • Child
  • Child, Preschool
  • Clinical Trials as Topic
  • Disease Management
  • Drugs, Investigational / therapeutic use
  • Enzyme Activation
  • Female
  • Forecasting
  • Gene Expression Profiling / methods
  • Humans
  • Male
  • Middle Aged
  • Molecular Targeted Therapy
  • Oncogene Proteins, Fusion / drug effects
  • Oncogene Proteins, Fusion / genetics
  • Oncogene Proteins, Fusion / physiology
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / classification
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / diagnosis*
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / drug therapy
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics
  • Prognosis
  • Protein Kinase Inhibitors / pharmacology
  • Protein Kinase Inhibitors / therapeutic use
  • Protein Kinases / drug effects
  • Protein Kinases / genetics
  • Protein Kinases / physiology
  • Receptors, Cytokine / drug effects
  • Receptors, Cytokine / genetics
  • Receptors, Cytokine / physiology
  • Sex Distribution
  • Signal Transduction / drug effects
  • Young Adult

Substances

  • Antineoplastic Agents
  • Drugs, Investigational
  • Oncogene Proteins, Fusion
  • Protein Kinase Inhibitors
  • Receptors, Cytokine
  • Protein Kinases