Intrinsic abnormalities of lymphocyte counts in children with Down syndrome
Intrinsic abnormalities of lymphocyte counts in children with Down syndrome
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DOI：
10.1016/j.jpeds.2005.07.022
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发表时间：
2005-12-01
影响因子：
5.1
通讯作者：
De Vries, E
中科院分区：
文献类型：
--
作者：
De Hingh, YCM;Van Der Vossen, PW;De Vries, E
目的唐氏综合征(DS)与感染、血液系统恶性肿瘤和自身免疫性疾病的发生率增加相关，提示免疫缺陷是DS的一个重要组成部分，是导致发病率和死亡率增加的重要原因。为了进一步了解DS儿童的免疫缺陷，我们测定了一大组DS儿童主要淋巴细胞亚群的绝对计数。研究设计在一大组DS儿童(n=96)中，用裂解全血三色免疫表型法检测主要淋巴细胞亚群的绝对数。结果在无DS的健康儿童中，出生后最初几年的T、B淋巴细胞的原发扩增被严重抑制。随着时间的推移，T淋巴细胞亚群逐渐趋于正常，而B淋巴细胞数量持续严重下降，88%下降到正常的第10百分位以下，61%下降到正常值的第5百分位以下。结论T、B淋巴细胞扩张性减弱是DS患者适应性免疫系统功能紊乱的内在表现，不是性早衰的反映。胸腺的改变被描述为DS可以解释T淋巴细胞数量的减少，但不能解释这些儿童中出现的显著的B淋巴细胞减少。
Objective Down syndrome (DS) is associated with an increased frequency of infections, hematologic malignancies, and autoimmune diseases, suggesting that immunodeficiency is an integral part of DS that contributes significantly to the observed increased morbidity and mortality. We determined the absolute counts of the main lymphocyte populations in a large group of DS children to gain further insight into this immunodeficiency.Study design In a large group of children with DS (n = 96), the absolute numbers of the main lymphocyte subpopulations were determined with 3-color immunophenotyping using the lysed whole-blood method. The results were compared with previously published data in healthy children without DS.Results In healthy children with DS, the primary expansion of T and B lymphocytes seen in healthy children without DS in the first years of life was severely abrogated. The T-lymphocyte subpopulation counts gradually reached more normal levels with time, whereas the B-lymphocyte population remained severely decreased, with 88% of values falling below the 10th percentile and 61% below the 5th percentile of normal.Conclusions The diminished expansion of T and B lymphocytes strongly suggests that a disturbance in the adaptive immune system is intrinsically present in DS and is not a reflection of precocious aging. Thymic alterations have been described in DS that could explain the decreased numbers of T lymphocytes, but not the striking B lymphocytopenia, seen in these children.