Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)

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Mann, G. et al.

Acute lymphoblastic leukemia with t(4;11) in children 1 year and older: The 'big sister' of the infant disease?

The t(4;11)-positive acute lymphoblastic leukemia (ALL) is a rare disease in children above the age of 1 year. We studied the clinical and biological characteristics in 32 consecutively diagnosed childhood cases (median age 10.0 years, range 1.0-17.1 years). Immunophenotyping revealed a pro-B and a pre-B stage in 24 and eight cases, respectively. IGH genes were rearranged in 84% of leukemias with a predominance of incomplete DJ(H) joints. Whereas IGK-Kde and TCRD rearrangements were rare, TCRG rearrangements were present in 50% of cases and involved mainly Vgamma11 or Vgamma9 together with a Jgamma1.3./2.3 gene segment, an unusual combination among t(4;11)-negative B-cell precursor ALL. Oligoclonality was found in about 30% as assessed by heterogeneous IGH and TCRG rearrangements. Our data are in line with transformation of a precursor cell at an early stage of B-cell development but retaining the potential to differentiate to the pre-B cell stage in vivo. Although a distinct difference between infant and older childhood cases with t(4;11) became evident, no age-related biological features were found within the childhood age group. In contrast to infants with t(4;11)-positive ALL, childhood cases had a relatively low cumulative incidence of relapse of 25% at 3.5 years with BFM-based high-risk protocols.Leukemia (2007) 21, 642-646. doi:10.1038/sj.leu.2404577; published online 8 February 2007.[1]

References

Acute lymphoblastic leukemia with t(4;11) in children 1 year and older: The 'big sister' of the infant disease? Mann, G., Cazzaniga, G., van der Velden, V.H., Flohr, T., Csinady, E., Paganin, M., Schrauder, A., Dohnal, A.M., Schrappe, M., Biondi, A., Gadner, H., van Dongen, J.J., Panzer-Grümayer, E.R. Leukemia (2007) [Pubmed]

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