Early T-cell precursor acute lymphoblastic leukaemia

Curr Opin Hematol. 2013 Jul;20(4):369-73. doi: 10.1097/MOH.0b013e3283623c61.


Purpose of review: Early T-cell precursor (ETP) leukaemias have been recently recognized as a form of T-cell acute lymphoblastic leukaemia (T-ALL) with a poor prognosis. The purpose of this review is to outline the most recent advances in the biology, genetics and prognostic significance of this aggressive disease.

Recent findings: Detailed immunophenotypic analyses have defined ETP T-ALLs as a distinct group of T-ALL with a poor prognosis. Transcriptionally, ETP T-ALLs and early immature T-ALLs, a broader group of tumours characterized by very early arrest in T-cell differentiation, are most related to haematopoietic stem cells and myeloid progenitors. Consistently, these leukaemias show lower frequencies of prototypical T-ALL lesions such as CDKN2A/B deletions and activating mutations in NOTCH1 and show a higher prevalence of mutations typically associated with the pathogenesis of acute myeloid leukaemias (AMLs).

Summary: ETP and early immature T-ALLs are characterized by a very early differentiation arrest and show unique genetic and transcriptional features that overlap both with T-ALL and with AML. Given the unique biology and poor prognosis associated with the ETP T-ALL group, there is an urgent need of new tailored therapeutic strategies for the treatment of this disease.

Publication types

  • Review

MeSH terms

  • Biomarkers, Tumor / metabolism
  • Gene Expression Profiling
  • Humans
  • Immunophenotyping
  • Precursor T-Cell Lymphoblastic Leukemia-Lymphoma* / genetics
  • Precursor T-Cell Lymphoblastic Leukemia-Lymphoma* / immunology
  • Precursor T-Cell Lymphoblastic Leukemia-Lymphoma* / metabolism
  • Prognosis
  • T-Lymphocytes / immunology
  • T-Lymphocytes / metabolism
  • Transcription, Genetic


  • Biomarkers, Tumor