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迟发性低丙种球蛋白血症的两种免疫上不同的形式。

Two immunologically distinct forms of late-onset hypogammaglobulinaemia.

作者信息

Platts-Mills T A, De Gast G C, Webster A D, Asherson G L, Wilkins S R

出版信息

Clin Exp Immunol. 1981 May;44(2):383-8.

Abstract

Most patients with late-onset bypogammaglobulinaemia have normal numbers of B cells. In addition, some of these patients have been shown to have circulating T cells which suppress immunolobulin production in vitro. From our present studies on a large series of patients it appears that these two findings distinguish two separate groups of patients. The majority of the patients have circulating B cells which are 'immature' in the sense that they produce IgM but very little IgG or IgA in vitro. These patients also fail to produce IgA in vivo but their T cells show normal function in vitro and normal T cell markers. Patients with the second form of the disease, including those with associated thymoma, have very few circulating B cells, and relatively preserved IgA production in vivo. In our series it was only this minority of patients whose T cells showed abnormal markers and increased suppressor activity in vitro.

摘要

大多数迟发性低丙种球蛋白血症患者的B细胞数量正常。此外,已证实其中一些患者有循环T细胞,这些T细胞在体外可抑制免疫球蛋白的产生。从我们目前对大量患者的研究来看,这两个发现似乎区分了两组不同的患者。大多数患者有循环B细胞,从某种意义上说,这些B细胞是“不成熟的”,因为它们在体外产生IgM,但产生的IgG或IgA很少。这些患者在体内也不能产生IgA,但他们的T细胞在体外显示出正常功能和正常的T细胞标志物。患有第二种疾病形式的患者,包括那些伴有胸腺瘤的患者,循环B细胞很少,且体内IgA产生相对保留。在我们的系列研究中,只有这少数患者的T细胞在体外显示出异常标志物和增强的抑制活性。

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本文引用的文献

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Role of helper, suppressor and B-cell defects in the pathogenesis of the hypogammaglobulinemias.
N Engl J Med. 1978 Jul 27;299(4):172-8. doi: 10.1056/NEJM197807272990404.

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