Costa-Carvalho B T, Viana M A, Brunialti M K C, Kallas E G, Salomao R
Division of Allergy, Clinical Immunology and Rheumatology of the Department of Pediatrics, Federal University of Sao Paulo, Sao Paulo, Brazil.
Clin Exp Immunol. 2004 May;136(2):291-6. doi: 10.1111/j.1365-2249.2004.02446.x.
In this report we evaluated CD4(+) T, CD8(+) T and natural killer (NK) cell counts, the levels of naive/memory subsets within the CD4(+) T lymphocyte population, expression of CD38 on T lymphocytes, and CD4(+) and CD8(+) T cell cytokine production in two girls with hyper-IgM (HIM) syndrome. Both girls developed recurrent infections early in infancy, presenting a wide spectrum of clinical manifestations, with a strikingly different disease severity between them. CD4(+) T cell counts were low in both children (patient 1: 214 cells/mm(3) and patient 2: 392 cells/mm(3)), and the CD4/CD8 T cell ratio was 0.4 for patient 1, the patient with the more severe disease, and 1.4 for patient 2. NK cell numbers were low in patient 1 (60 cells/mm(3)) and borderline (286 cells/mm(3)) with regard to normal levels in patient 2. An imbalance of naive and memory/effector cell subsets was found in both girls, with the percentage of CD45RA(+) 27(+) (naive) CD4(+) T lymphocytes being 5.8 and 12.4 for patients 1 and 2, respectively. Expression of CD38 on the surface of T lymphocytes was low in patient 1. Detection of intracellular interferon (IFN)-gamma and tumour necrosis factor (TNF)-alpha in CD4(+) and CD8(+) T lymphocytes upon PMA-Io stimulus was preserved in both children. In conclusion, we found low numbers of CD4(+) T lymphocytes and a dramatic redistribution of naive and memory/effector CD4(+) T lymphocytes in two girls with non-X-linked HIM syndrome. Furthermore, we found low expression of CD38 on T lymphocytes and low numbers of NK cells in the patient with the more severe disease, indicating a possible role for these cells in the pathogenesis of this immunodeficiency.
在本报告中，我们评估了两名高IgM（HIM）综合征女孩的CD4(+) T细胞、CD8(+) T细胞和自然杀伤（NK）细胞计数，CD4(+) T淋巴细胞群体中初始/记忆亚群的水平，T淋巴细胞上CD38的表达，以及CD4(+)和CD8(+) T细胞细胞因子的产生。两名女孩均在婴儿期早期出现反复感染，临床表现多样，且疾病严重程度差异显著。两名儿童的CD4(+) T细胞计数均较低（患者1：214个细胞/mm(3)，患者2：392个细胞/mm(3)），病情较重的患者1的CD4/CD8 T细胞比值为0.4，患者2的该比值为1.4。患者1的NK细胞数量较低（60个细胞/mm(3)），而患者2的NK细胞数量接近正常水平（286个细胞/mm(3)）。在两名女孩中均发现初始细胞和记忆/效应细胞亚群失衡，患者1和患者2的CD45RA(+) 27(+)（初始）CD4(+) T淋巴细胞百分比分别为5.8%和12.4%。患者1的T淋巴细胞表面CD38表达较低。在两名儿童中，经佛波酯-离子霉素（PMA-Io）刺激后，CD4(+)和CD8(+) T淋巴细胞内干扰素（IFN）-γ和肿瘤坏死因子（TNF）-α的检测结果均正常。总之，我们发现两名非X连锁HIM综合征女孩的CD4(+) T淋巴细胞数量较低，且初始和记忆/效应CD4(+) T淋巴细胞发生显著重新分布。此外，我们发现病情较重的患者T淋巴细胞上CD38表达较低，NK细胞数量较少，表明这些细胞可能在这种免疫缺陷的发病机制中起作用。
