Lindor K D, Wiesner R H, Katzmann J A, LaRusso N F, Beaver S J
Dig Dis Sci. 1987 Jul;32(7):720-5. doi: 10.1007/BF01296138.
As an initial step in testing the hypothesis that immunoregulatory abnormalities are important in the pathogenesis of primary sclerosing cholangitis, we determined the number and percentage of lymphocyte subsets in the peripheral blood of 33 patients with primary sclerosing cholangitis. In these patients, when compared with normal and diseased controls, there was a significant reduction in the total number of circulating T cells because of a disproportionate decrease in Leu-2a (suppressor/cytotoxic) cells. This decrease resulted in a significantly increased ratio of Leu-3a to Leu-2a cells. Patients with cirrhosis had significantly higher Leu-3a/Leu-2a (helper/suppressor) ratios than did noncirrhotic patients; both disease groups, however, had ratios that were significantly higher than controls. The number and percentage of B cells were significantly increased. Alterations in the percentage of B cells correlated significantly with histologic stage and concentrations of gamma globulin, serum IgG, and bilirubin. We conclude that these abnormalities are suggestive of a defect in immunoregulation in primary sclerosing cholangitis, which is not secondary to advanced liver disease alone and appears to be independent of chronic ulcerative colitis or obstructive jaundice.
作为检验免疫调节异常在原发性硬化性胆管炎发病机制中起重要作用这一假说的第一步，我们测定了33例原发性硬化性胆管炎患者外周血中淋巴细胞亚群的数量和百分比。在这些患者中，与正常对照和患病对照相比，循环T细胞总数显著减少，原因是Leu - 2a（抑制/细胞毒性）细胞不成比例地减少。这种减少导致Leu - 3a与Leu - 2a细胞的比例显著增加。肝硬化患者的Leu - 3a/Leu - 2a（辅助/抑制）比值显著高于非肝硬化患者；然而，两个疾病组的比值均显著高于对照组。B细胞的数量和百分比显著增加。B细胞百分比的改变与组织学分期以及γ球蛋白、血清IgG和胆红素浓度显著相关。我们得出结论，这些异常提示原发性硬化性胆管炎存在免疫调节缺陷，这并非仅继发于晚期肝病，且似乎独立于慢性溃疡性结肠炎或梗阻性黄疸。
