Abstract
Because of their increased malignancies, autoimmune diseases, and infections, patients with Down syndrome (DS) show features of immunodeficiency. The DS thymus and T lymphocyte subsets have indeed proven to be different, and this has been interpreted as precocious aging. Our study on T lymphocyte subpopulations in DS shows that the normal expansion of naive helper (CD4+CD45RA+) and cytotoxic (CD8+CD45RA+CD27+) T lymphocytes is lacking in the first years of life; this is more logically explainable with an intrinsic T lymphocyte defect. Furthermore, memory cell numbers are not different from age-matched controls (AMC), which does not support the hypothesis of precocious aging. Although the absolute numbers of T lymphocyte subpopulations approach AMC levels toward adulthood, the persistent clinical problems suggest that these cells may not function optimally. However, the clinical picture does not fit severe T lymphocyte deficiency. The latter concept is also supported by our finding that cytomegalovirus (CMV)-seropositive DS children show similar numbers of terminally differentiated cytotoxic T lymphocytes when compared with healthy children, not increased numbers as are seen in immunocompromised hosts.
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Abbreviations
- AMC:
-
Age-matched control
- BD:
-
Becton Dickinson
- CMV:
-
Cytomegalovirus
- DS:
-
Down syndrome
- NK:
-
Natural killer
- PE:
-
Phycoerythrin
- PE-Cy5:
-
PE-cyanin 5
- Tc:
-
Cytotoxic T
- Th:
-
Helper T
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Acknowledgements
We thank patients and parents for their cooperation with the study and Mr. Jeroen Ooms for preparing the figures.
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Supported by the JBZ Research Fund and the Irene Foundation.
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Kusters, M., Gemen, E., Verstegen, R. et al. Both Normal Memory Counts and Decreased Naive Cells Favor Intrinsic Defect Over Early Senescence of Down Syndrome T Lymphocytes. Pediatr Res 67, 557–562 (2010). https://doi.org/10.1203/PDR.0b013e3181d4eca3
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DOI: https://doi.org/10.1203/PDR.0b013e3181d4eca3
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