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

2008; Wiley; Volume: 143; Issue: 2 Linguagem: Inglês

10.1111/j.1365-2141.2008.07314.x

1365-2141

Pieter Van Vlierberghe, Rob Pieters, H. Berna Beverloo, Jules P.P. Meijerink,

Epigenetics and DNA Methylation

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.