Age-related expansion of functionally inefficient cells with markers of
natural killer activity in Down's syndrome
A Cossarizza, C Ortolani, E Forti, G Montagnani, R Paganelli, M Zannotti, M Marini, D Monti and C Franceschi
Institute of General Pathology, University of Modena School of Medicine,
Italy.
Peripheral blood lymphocyte subsets of two groups of patients affected by
Down's syndrome (DS), ie, 28 children and nine adults of relatively
advanced age (greater than 34 years), were investigated and compared with
those of age- and sex-matched healthy controls (13 children and 20 adults).
Particular attention was devoted to cells with markers of natural killer
(NK) activity. Double- and triple-color cytofluorimetric analysis was used
to better characterize the phenotypic features of the different subsets.
Apart from a reduced number of T lymphocytes (CD3+) in DS children and of B
lymphocytes (CD19+) in both DS groups, the major alteration we found was a
marked age-related increase of the percentage of cells bearing markers
associated with NK activity, such as CD16, CD56, and CD57. These DS cells
were apparently severely defective as far as their function was concerned,
because NK activity was significantly reduced in comparison with
age-matched controls, but still capable of responding to cytokines such as
interleukin-2, interferon-beta, and interferon-gamma, and to the modulation
of lytic activity exerted by the anti-CD16 monoclonal antibody. On the
whole, our data stress the importance of studying DS subjects of different
ages to fully appreciate the immunologic derangement characteristic of this
syndrome.
Volume 77,
Issue 6,
pp. 1263-1270,
03/15/1991
Copyright © 1991 by The American Society of Hematology
