Deficiency of T helper cells in transient hypogammaglobulinemia of infancy.
Deficiency of T helper cells in transient hypogammaglobulinemia of infancy.
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婴儿期短暂性低丙种球蛋白血症中 T 辅助细胞缺乏。
DOI：
10.1056/nejm198111263052202
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发表时间：
1981
期刊：
影响因子：
--
通讯作者：
R. Geha
中科院分区：
文献类型：
--
作者：
R. Siegel;T. Issekutz;J. Schwaber;F. Rosen;R. Geha
我们研究了 17 名患有婴儿期短暂性低丙种球蛋白血症的患者，以确定导致这种疾病的免疫缺陷。这些患者的循环 B 细胞数量正常，B 细胞在受到 Epstein-Barr 病毒（一种直接 B 细胞激活剂）刺激时合成免疫球蛋白的能力也正常。然而，B 细胞响应商陆丝裂原（一种 T 细胞依赖性 B 细胞激活剂）合成 IgG 的能力受到抑制。对培养的淋巴细胞进行的实验表明，这些患者中不存在过度的抑制细胞活性，但他们的 T 细胞缺乏向正常父母的 B 细胞提供帮助的能力。发现 T4 阳性 (T4+) 辅助细胞数量不足。从该疾病中康复的患者具有正常数量的 T4+ 辅助细胞。我们的结果表明，辅助性 T 细胞的数量和功能缺陷是婴儿期短暂性低丙种球蛋白血症中 IgG 生成缺陷的基础。
We studied 17 patients with transient hypogammaglobulinemia of infancy to define the immunologic defect responsible for this disorder. The number of circulating B cells in these patients was normal, as was the ability of the B cells to synthesize immunoglobulins when stimulated with Epstein-Barr virus, a direct B-cell activator. However, the capacity of the B cells to synthesize IgG in response to pokeweed mitogen, a T-cell-dependent B-cell activator, was depressed. Experiments with cultured lymphocytes indicated that excess suppressor-cell activity was not present in these patients, but that their T cells were deficient in providing help to B cells from their normal parents. A numerical deficiency in T4-positive (T4+) helper cells was found. Patients who had recovered from the disorder had a normal number of T4+ helper cells. Our results indicate that a numerical, as well as a functional, deficiency in helper T cells underlies the deficiency in IgG production in transient hypogammaglobulinemia of infancy.
与 T4 诱导 T 细胞功能丧失相关的免疫缺陷。
DOI：
10.1056/nejm198104023041403
发表时间：
1981
期刊：
The New England journal of medicine
影响因子：
--
作者：
Reinherz,EL;Geha,R;Wohl,ME;Morimoto,C;Rosen,FS;Schlossman,SF
通讯作者：
Schlossman,SF