Cossarizza A, Monti D, Montagnani G, Ortolani C, Masi M, Zannotti M, Franceschi C
Department of Immunology, University of Modena School of Medicine, Italy.
Am J Med Genet Suppl. 1990;7:213-8. doi: 10.1002/ajmg.1320370743.
Phenotype and proliferative ability of peripheral blood lymphocytes from 15 noninstitutionalized children affected with Down Syndrome (DS), in apparently good health, were studied and compared with those of 16 healthy control children of the same age. A complex derangement of all the major peripheral blood cell subsets, i.e., B cells, T cells, and natural killer (NK) cells, was present in DS children. A significant decrease of the absolute number of circulating lymphocytes, a marked and significant decrease of B lymphocyte absolute number and percentage, and dramatic modifications of the T-cell subsets were observed. The absolute number of CD4+ cells was significantly decreased, whereas CD8+ cells increased significantly in percentage but not in absolute number. A derangement of cells bearing markers associated with NK activity, such as CD57, CD16, and CD56, was observed. Among the most important alterations, the presence of a high number of CD57+, CD16- cells, of CD57+, CD8+ lymphocytes, and of CD3+, CD56+ lymphocytes was seen. Many of these alterations are similar to those characteristic of chromosomally normal subjects of advanced age. The hypothesis that the reduced thymic endocrine activity and the zinc deficiency characteristic of DS are responsible for the derangement of T and NK subsets is discussed.
对15名非收容机构照料的、外表健康的唐氏综合征(DS)患儿的外周血淋巴细胞的表型和增殖能力进行了研究,并与16名同龄健康对照儿童的外周血淋巴细胞进行了比较。DS患儿存在所有主要外周血细胞亚群,即B细胞、T细胞和自然杀伤(NK)细胞的复杂紊乱。观察到循环淋巴细胞绝对数量显著减少,B淋巴细胞绝对数量和百分比显著降低,以及T细胞亚群的显著改变。CD4+细胞的绝对数量显著减少,而CD8+细胞的百分比显著增加,但绝对数量未增加。观察到带有与NK活性相关标志物的细胞,如CD57、CD16和CD56的紊乱。在最重要的改变中,发现存在大量CD57+、CD16-细胞,CD57+、CD8+淋巴细胞,以及CD3+、CD56+淋巴细胞。其中许多改变与高龄染色体正常受试者的特征相似。讨论了DS特有的胸腺内分泌活性降低和锌缺乏导致T和NK亚群紊乱的假说。