Roder J C, Haliotis T, Laing L, Kozbor D, Rubin P, Pross H, Boxer L A, White J G, Fauci A S, Mostowski H, Matheson D S
Immunology. 1982 Jul;46(3):555-60.
Spontaneous natural killer (NK) activity and antibody-dependent cellular cytotoxicity (ADCC) of blood lymphocytes against five human tumour cell lines (K562, Molt-4, HL-60, Chang, Daudi) and three mouse tumour lines (YAC, P815, RBL-5) were ten- to 100-fold lower than normal in six patients with Chediak-Higashi (CH) disease. NK and ADCC were defective at 4 hr, and less so at 18 hr. The NK activity in normals and CH patients was mediated in part by FcR+, E- effector cells. ADCC against human erythrocytes was normal in CH patients, as were lectin-dependent cytolysis and mixed lymphocyte proliferative responses. Phagocytosis of antibody-coated ox erythrocytes was normal in CH patients as well. These observations confirm that the CH syndrome is associated with a profound and selective defect in NK and ADCC activity against tumour cells, whereas other mononuclear cell-mediated functions are normal.
在6例患有切-东(CH)综合征的患者中,血液淋巴细胞对5种人类肿瘤细胞系(K562、Molt-4、HL-60、Chang、Daudi)和3种小鼠肿瘤细胞系(YAC、P815、RBL-5)的自发自然杀伤(NK)活性及抗体依赖性细胞毒性(ADCC)比正常人低10至100倍。NK和ADCC在4小时时存在缺陷,在18小时时缺陷程度较轻。正常人和CH患者的NK活性部分由FcR⁺、E⁻效应细胞介导。CH患者针对人红细胞的ADCC正常,凝集素依赖性细胞溶解和混合淋巴细胞增殖反应也正常。CH患者对抗体包被的牛红细胞的吞噬作用也正常。这些观察结果证实,CH综合征与针对肿瘤细胞的NK和ADCC活性存在深刻且选择性的缺陷相关,而其他单核细胞介导的功能则正常。