Factors involved in the generation of memory CD8+ T cells in patients with X-linked lymphoproliferative disease (XLP)

Authors

  • L. Belmonte,

    1. Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Buenos Aires, Argentina, and
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    • These authors contributed equally to this study.

  • C. Parodi,

    1. Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Buenos Aires, Argentina, and
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    • These authors contributed equally to this study.

  • P. Baré,

    1. Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Buenos Aires, Argentina, and
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  • A. Malbrán,

    1. Departamento de Alergia e Inmunología, Hospital Británico, Buenos Aires, Argentina
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  • B. Ruibal-Ares,

    1. Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Buenos Aires, Argentina, and
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  • María M. de E. De Bracco

    Corresponding author
    1. Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Buenos Aires, Argentina, and
      Dr María M. E. de Bracco, Inmunología, Instituto de Investigaciones, Hematológicas (IIHEMA), Academia Nacional de Medicina, P. de Melo 3081, 1425, Buenos Aires, Argentina.
      E-mail: mebracco@hematologia.anm.edu.ar
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Dr María M. E. de Bracco, Inmunología, Instituto de Investigaciones, Hematológicas (IIHEMA), Academia Nacional de Medicina, P. de Melo 3081, 1425, Buenos Aires, Argentina.
E-mail: mebracco@hematologia.anm.edu.ar

Summary

We have analysed the phenotype of T lymphocytes in two X-linked lymphoproliferative disease (XLP) patients with the same SH2D1A mutation differing in initial exposure to Epstein–Barr virus (EBV) and treatment. While memory T lymphocytes (with low CCR7 and CD62L expression) prevailed in both XLP patients, in patient 9, who developed acute infectious mononucleosis (AIM) and received B cell ablative treatment, the predominant phenotype was that of late effector CD8 T cells (CD27, CD28, CCR7, CD62L, CD45 RA+, perforin+), while in patient 4 (who did not suffer AIM) the prevalent phenotype of CD8 T lymphocytes was similar to that of normal controls (N) or to that of adult individuals who recovered from AIM: CD27+ , CD28+, CCR7, CD62L, CD45 RO+ and perforin. CD57 expression (related to senescence) was also higher in CD8 T cells from patient 9 than in patient 4, AIM or N. Persistently high EBV viral load was observed in patient 9. The results obtained from this limited number of XLP patients suggest that events related to the initial EBV encounter (antigen load, treatment, cytokine environment) may have more weight than lack of SH2D1A in determining the long-term differentiation pattern of CD8 memory T cells.

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