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.

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Keywords Acute leukaemia, Biological aspects, Pathology
Persistent URL dx.doi.org/10.1111/j.1365-2141.2008.07314.x, hdl.handle.net/1765/29052
Citation
van Vlierberghe, P, Pieters, R, Beverloo, H.B, & Meijerink, J.P.P. (2008). Molecular-genetic insights in paediatric T-cell acute lymphoblastic leukaemia. British Journal of Haematology, 143(2), 153–168. doi:10.1111/j.1365-2141.2008.07314.x