Recent Advances in the Biology and Treatment of T Cell Acute Lymphoblastic Leukemia

Curr Hematol Malig Rep. 2018 Aug;13(4):265-274. doi: 10.1007/s11899-018-0455-9.

Abstract

Purpose of review: This article provides an overview of the current knowledge regarding the biology and treatment of T cell acute lymphoblastic leukemia (T-ALL) and highlights the most recent findings in this field over the past 5 years.

Recent findings: Remarkable progress has been made in the genomic landscape of T-ALL over the past few years. The discovery of activating mutations of NOTCH1 and FBXW7 in a majority of patients has been a seminal observation, with several early phase clinical trials currently exploring these as potential therapeutic targets. Characterization of early T cell precursor ALL, incorporation of minimal residual disease assessment into therapeutic protocols, and use of pediatric-intensive regimens along with judicious use of allogeneic HCT have significantly improved risk stratification and treatment outcomes. Improved risk stratification and the use of novel targeted therapies based on recent genomic discoveries are expected to change the therapeutic landscape of T-ALL and hopefully improve the outcomes of this historically poor prognosis disease.

Keywords: Early T cell precursor acute lymphoblastic leukemia; Minimal residual disease; Nelarabine; T cell acute lymphoblastic leukemia; Targeted therapies.

Publication types

  • Review

MeSH terms

  • F-Box-WD Repeat-Containing Protein 7* / genetics
  • F-Box-WD Repeat-Containing Protein 7* / metabolism
  • Humans
  • Mutation*
  • Neoplasm Proteins* / genetics
  • Neoplasm Proteins* / metabolism
  • Precursor T-Cell Lymphoblastic Leukemia-Lymphoma* / genetics
  • Precursor T-Cell Lymphoblastic Leukemia-Lymphoma* / metabolism
  • Precursor T-Cell Lymphoblastic Leukemia-Lymphoma* / therapy
  • Receptor, Notch1* / genetics
  • Receptor, Notch1* / metabolism

Substances

  • F-Box-WD Repeat-Containing Protein 7
  • FBXW7 protein, human
  • NOTCH1 protein, human
  • Neoplasm Proteins
  • Receptor, Notch1