Molecular-genetic insights in paediatric T-cell acute lymphoblastic leukaemia

Br J Haematol. 2008 Oct;143(2):153-68. doi: 10.1111/j.1365-2141.2008.07314.x. Epub 2008 Aug 7.


Paediatric T-cell acute lymphoblastic leukaemia (T-ALL) is an aggressive malignancy of thymocytes that accounts for about 15% of ALL cases and for which treatment outcome remains inferior compared to B-lineage acute leukaemias. In T-ALL, leukemic transformation of maturating thymocytes is caused by a multistep pathogenesis involving numerous genetic abnormalities that drive normal T-cells into uncontrolled cell growth and clonal expansion. This review provides an overview of the current knowledge on onco- and tumor suppressor genes in T-ALL and suggests a classification of these genetic defects into type A and type B abnormalities. Type A abnormalities may delineate distinct molecular-cytogenetic T-ALL subgroups, whereas type B abnormalities are found in all major T-ALL subgroups and synergize with these type A mutations during T-cell pathogenesis.

Publication types

  • Review

MeSH terms

  • Cell Cycle / genetics
  • Cell Differentiation / genetics
  • Child
  • Gene Rearrangement, T-Lymphocyte
  • Genes, Homeobox*
  • Humans
  • Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / classification
  • Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / genetics*
  • Proto-Oncogenes*
  • Receptors, Antigen, T-Cell / genetics*
  • Signal Transduction / genetics*


  • Receptors, Antigen, T-Cell