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成人选择性IgM缺乏症:外周血淋巴细胞的表型和功能改变特征

Selective IgM deficiency in adults: phenotypically and functionally altered profiles of peripheral blood lymphocytes.

作者信息

Ohno T, Inaba M, Kuribayashi K, Masuda T, Kanoh T, Uchino H

机构信息

Department of Internal Medicine, Faculty of Medicine, Kyoto University, Japan.

出版信息

Clin Exp Immunol. 1987 Jun;68(3):630-7.

Abstract

Peripheral blood lymphocytes from four patients with selective IgM deficiency were examined phenotypically and functionally. Although B cell subpopulations determined by surface immunoglobulins were within normal or nearly normal range, T8+ cells were significantly increased and T4/T8 ratios were inverted in three patients. IgM specific hyporesponsiveness in the PWM-driven immunoglobulin production system was observed in all four patients. Ia-like antigen positive T cells were increased in two patients; both had increased Leu2a+ Leu15+ suppressor-effector cells. In addition, Leu3a+ Leu8+ suppressor-inducer cells were increased in one of these two patients. Excessive (either IgM-specific or isotype non-specific) suppressor activity of T cells and IgM specific hyporesponsiveness of non-T cells were observed in these two patients in the recombination plaque assay. Although these results showed the complexity of the pathogenesis of this syndrome, they suggested that suppressor-associated T cells may play a role in some patients with selective IgM deficiency.

摘要

对4例选择性IgM缺乏症患者的外周血淋巴细胞进行了表型和功能检测。尽管通过表面免疫球蛋白确定的B细胞亚群在正常或接近正常范围内,但3例患者的T8 +细胞显著增加,T4/T8比值倒置。在所有4例患者中均观察到在PWM驱动的免疫球蛋白产生系统中IgM特异性低反应性。2例患者中Ia样抗原阳性T细胞增加;两者的Leu2a + Leu15 +抑制效应细胞均增加。此外,在这2例患者中的1例中Leu3a + Leu8 +抑制诱导细胞增加。在这2例患者的重组斑试验中观察到T细胞的过度(IgM特异性或同种型非特异性)抑制活性和非T细胞的IgM特异性低反应性。尽管这些结果表明该综合征发病机制的复杂性,但提示抑制相关T细胞可能在某些选择性IgM缺乏症患者中起作用。

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