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X连锁淋巴增殖综合征患者存在自然杀伤活性缺陷,但保留淋巴细胞介导的抗体依赖性细胞毒性。

Defective natural killing activity but retention of lymphocyte-mediated antibody-dependent cellular cytotoxicity in patients with the X-linked lymphoproliferative syndrome.

作者信息

Argov S, Johnson D R, Collins M, Koren H S, Lipscomb H, Purtilo D T

出版信息

Cell Immunol. 1986 Jun;100(1):1-9. doi: 10.1016/0008-8749(86)90001-8.

DOI:10.1016/0008-8749(86)90001-8
PMID:3742598
Abstract

Cellular mediated immune responses in vitro of six males with X-linked lymphoproliferative syndrome (XLP) were investigated. Impaired natural killing (NK) activity compared to seven controls (P less than 0.001) and interferon-alpha activation (P less than 0.011) were detected. However, normal numbers of lymphocytes expressing NK-associated antigens (Leu-7 and MO1) and large granular lymphocytes (LGL) were observed in all patients. Normal lymphocyte-mediated antibody-dependent cellular cytotoxicity (ADCC) (P less than 0.531) was found. NK and ADCC activities have been regarded as being mediated by the same subsets of lymphocytes, however, results of this study do not support this hypothesis: lymphocytes with impaired NK but normal ADCC functions (NK-/K+) were found in males with XLP.

摘要

对6名患有X连锁淋巴增殖综合征(XLP)的男性患者的细胞介导免疫反应进行了体外研究。与7名对照相比,检测到自然杀伤(NK)活性受损(P<0.001)和α干扰素激活受损(P<0.011)。然而,在所有患者中均观察到表达NK相关抗原(Leu-7和MO1)的淋巴细胞和大颗粒淋巴细胞(LGL)数量正常。发现淋巴细胞介导的抗体依赖性细胞毒性(ADCC)正常(P<0.531)。NK和ADCC活性被认为是由相同的淋巴细胞亚群介导的,然而,本研究结果不支持这一假设:在患有XLP的男性中发现了NK功能受损但ADCC功能正常的淋巴细胞(NK-/K+)。

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