Tomiyama J, Ninomiya H, Abe T
Division of Haematology, University of Tsukuba, Ibaraki, Japan.
Br J Haematol. 1990 Dec;76(4):540-4. doi: 10.1111/j.1365-2141.1990.tb07913.x.
We investigated the complement-susceptibility of paroxysmal nocturnal haemoglobinuria (PNH) lymphocytes in relation to their dysfunction. When assessed by complement-mediated lysis induced by monoclonal antibodies (CD5 or CD20) and rabbit complement, the complement-susceptibility of lymphocytes from patients with PNH, both CD5+ (T cells) and CD20+ (B cells), was greater than that from controls (P less than 0.001). This susceptibility was further enhanced, both in normal controls (P less than 0.01) and in patients with PNH (P less than 0.001), when the activity of decay-accelerating factor (DAF) on the lymphocytes was blocked with anti-DAF monoclonal antibody. DAF amounts in mononuclear cells (MNC) from patients with PNH, measured by an enzyme-linked immunosorbent assay (ELISA), were lower than those of the controls (P less than 0.001). The expressions of DAF on T cells and on B cells from patients with PNH were significantly decreased (P less than 0.05 in T cells: P less than 0.01 in B cells). MNC from patients with PNH responded less to phytohaemagglutinin (PHA) and concanavalin A (Con A) than MNC from the controls (P less than 0.001). In contrast, the responses of PNH MNC to poke weed mitogen (PWM) or lipopolysaccharide (LPS) were not impaired. MNC from a normal donor preincubated with anti-DAF became less responsive to PHA or Con A. We conclude that PNH lymphocytes show enhanced complement-susceptibility and that they are involved in their own expression of DAF as well as in other types of peripheral blood cells. The results of the responses to various lectins suggest the dysfunction of T cells in PNH.
我们研究了阵发性夜间血红蛋白尿（PNH）淋巴细胞的补体敏感性与其功能障碍的关系。当通过单克隆抗体（CD5或CD20）和兔补体诱导的补体介导的细胞溶解进行评估时，PNH患者的淋巴细胞，无论是CD5 +（T细胞）还是CD20 +（B细胞），其补体敏感性均高于对照组（P <0.001）。当用抗衰变加速因子（DAF）单克隆抗体阻断淋巴细胞上的DAF活性时，正常对照组（P <0.01）和PNH患者（P <0.001）的这种敏感性均进一步增强。通过酶联免疫吸附测定（ELISA）测量，PNH患者单核细胞（MNC）中的DAF含量低于对照组（P <0.001）。PNH患者T细胞和B细胞上DAF的表达明显降低（T细胞中P <0.05；B细胞中P <0.01）。与对照组的MNC相比，PNH患者的MNC对植物血凝素（PHA）和刀豆球蛋白A（Con A）的反应较弱（P <0.001）。相反，PNH MNC对商陆有丝分裂原（PWM）或脂多糖（LPS）的反应未受损。用抗DAF预孵育的正常供体的MNC对PHA或Con A的反应性降低。我们得出结论，PNH淋巴细胞表现出增强的补体敏感性，并且它们参与自身DAF的表达以及其他类型的外周血细胞。对各种凝集素的反应结果表明PNH中T细胞功能障碍。
