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低丙种球蛋白血症患者白细胞对B细胞分化的抑制作用。

Suppression of B-cell differentiation by leukocytes from hypogammaglobulinemic patients.

作者信息

Siegal F P, Siegal M, Good R A

出版信息

J Clin Invest. 1976 Jul;58(1):109-22. doi: 10.1172/JCI108439.

Abstract

Leukocytes from patients with several forms of immunodeficiency characterized by apparently differing defects in B-lymphocyte maturation produced few or no plasmacytoid cells in vitro, and were capable of suppressing the generation of plasma cells in co-culture with cells of normal persons, in the presence of pokeweed mitogen. Such inhibition was commonly observed in cultures which included cells from patients with primary immunoglobulin deficiency, but was not seen to a significant degree in identical co-cultures of cells from normals. The suppression observed was not dependent upon mixed leukocyte culture reactivity. Both sheep erythrocyte-rosetting lymphocytes and adherent cells appeared to participate in these effects in some patients. In one patient with common variable immunodeficiency, but not in several others, removal of suppressing cells permitted the patient's remaining cells to differentiate into plasma cells in vitro. Because of the diverse syndromes in which suppression was observed, it is likely that, in at least some hypogammaglobulinemic patients, the suppression is secondary to the disease process rather than being the primary pathogenic mechanism.

摘要

来自患有几种形式免疫缺陷的患者的白细胞,其特征是B淋巴细胞成熟存在明显不同的缺陷,在体外产生很少或不产生浆细胞样细胞,并且在有商陆有丝分裂原存在的情况下,与正常人的细胞共培养时能够抑制浆细胞的产生。这种抑制在包括原发性免疫球蛋白缺乏症患者细胞的培养物中普遍观察到,但在正常人细胞的相同共培养物中未观察到显著程度的抑制。观察到的抑制不依赖于混合淋巴细胞培养反应性。在一些患者中,绵羊红细胞玫瑰花结形成淋巴细胞和贴壁细胞似乎都参与了这些效应。在一名常见可变免疫缺陷患者中,但在其他几名患者中未观察到,去除抑制细胞可使患者剩余的细胞在体外分化为浆细胞。由于观察到抑制作用的综合征多种多样,至少在一些低丙种球蛋白血症患者中,抑制作用很可能是疾病过程的继发结果,而不是主要的致病机制。

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