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Growth of large chromosomally abnormal T cell clones in ataxia telangiectasia patients is associated with translocation at 14q11

A model for other T cell neoplasia

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Summary

Human T cell malignancies often show chromosome breaks at 14q11, within the α chain locus of the human T cell antigen receptor, with translocation of the distal portion of 14 to one of several sites. In patients with ataxia telangiectasia (A-T) the majority of T cell chromosome translocations associated with this disorder appear to occur at the sites of the T cell antigen receptor genes 7p14, 7q35, and 14q11 and may result in clone formation. In three large proliferating A-T T cell clones we have observed (including one which became malignant) and in most T cell tumours reported, the clonal chromosome exchange involves one breakpoint at 14q11 with the second breakpoint occurring in a gene not involved in the immunoglobulin supergene family. Our observations on A-T patients confirm the suggestion that chromosome exchanges involving either t(7;14)(p14;q11), t(7;14)(q35;q11), inv(7) (p14q35), or t(7;7)(p14;q35) confer only a small proliferative advantage on T cells in vivo without the capacity for malignant transformation and that the potential for malignant change is not a feature of all these rearrangements, but is restricted to cells or clones with other chromosome exchanges.

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Authors and Affiliations

  1. Cancer Research Campaign Laboratories, Department of Cancer Studies, Medical School, B15 2TJ, Birmingham, UK

    R. J. Hollis, A. A. Kennaugh, S. V. Butterworth & A. M. R. Taylor

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  1. R. J. Hollis
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  2. A. A. Kennaugh
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  3. S. V. Butterworth
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  4. A. M. R. Taylor
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Hollis, R.J., Kennaugh, A.A., Butterworth, S.V. et al. Growth of large chromosomally abnormal T cell clones in ataxia telangiectasia patients is associated with translocation at 14q11. Hum Genet 76, 389–395 (1987). https://doi.org/10.1007/BF00272451

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  • DOI: https://doi.org/10.1007/BF00272451

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