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Acute Leukemias

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

Leukemia volume 21, pages 642–646 (2007)Cite this article

Abstract

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 DJH joints. Whereas IGK-Kde and TCRD rearrangements were rare, TCRG rearrangements were present in 50% of cases and involved mainly Vγ11 or Vγ9 together with a Jγ1.3./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.

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Acknowledgements

We thank OA Haas, A Leszl, T Lion, G Fazio and J Harbott for cytogenetic, RT-PCR and FISH data, Uli Pötschger for statistical analysis and Marion Zavadil for proofreading. This work was supported in part by the Österreichische Kinderkrebsforschung and private donations to the CCRI, by Fondazione Tettamanti, AIRC, Fondazione Città della Speranza and Fondazione Cariplo, Deutsche Krebshilfe (Grant 50-2698-Schr1) and German BMBF Competence Network Pediatric Oncology/Hematology (Grant 01 Gi 9963/2). It reflects a collaboration of members of the Biology Group of the International Berlin-Frankfurt-Münster (I-BFM) Group.

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Author notes

  1. G Mann and G Cazzaniga: These authors contributed equally to the paper.

Authors and Affiliations

  1. Department of Pediatric Hematology/Oncology, St. Anna Kinderspital, Vienna, Austria

    G Mann, H Gadner & E R Panzer-Grümayer

  2. Centro Ricerca Tettamanti, Pediatric Clinic, University of Milano Bicocca, Monza, Italy

    G Cazzaniga & A Biondi

  3. Department of Immunology, Erasmus MC, Rotterdam, The Netherlands

    V H J van der Velden & J J M van Dongen

  4. Department of Pediatrics, University Hospital Schleswig-Holstein, Kiel, Germany

    T Flohr, A Schrauder & M Schrappe

  5. Children's Cancer Research Institute CCRI, Vienna, Austria

    E Csinady, A M Dohnal, H Gadner & E R Panzer-Grümayer

  6. Laboratorio di Onco-Ematologia, Clinica Pediatrica, Università di Padova, Padova, Italy

    M Paganin

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Correspondence to E R Panzer-Grümayer.

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Mann, G., Cazzaniga, G., van der Velden, V. et al. Acute lymphoblastic leukemia with t(4;11) in children 1 year and older: The ‘big sister’ of the infant disease?. Leukemia 21, 642–646 (2007). https://doi.org/10.1038/sj.leu.2404577

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