Forastiero R R, Kordich L, Basilotta E, Carreras L O
University Institute of Biomedical Sciences, Favaloro Foundation, Buenos Aires, Argentina.
Blood Coagul Fibrinolysis. 1994 Aug;5(4):609-16.
Total protein S (tPS), free protein S (fPS) and C4b-binding protein (C4b-BP) were measured by immunological assays in 73 patients with antiphospholipid (aPL) antibodies, in order to determine whether the previously reported abnormalities in PS levels in this group of patients could be related to the presence of lupus anticoagulant (LA) or anticardiolipin (aCL) antibodies. As compared with the normal controls (n = 44), the authors found a significant decrease of tPS, fPS and C4b-BP in 45 LA(+)aCL(+) patients (P < 0.001), a decrease of tPS (P < 0.001), fPS and C4b-BP (P < 0.01) in eight LA(-)aCL(+) patients and a decrease of only fPS (P < 0.05) in 20 LA(+)aCL(-) patients. There was no difference in the levels of tPS, fPS and C4b-BP between LA(+)aCL(+) and LA(-)aCL(+) patients. In contrast, the LA(+)aCL(+) patients had lower values of tPS, fPS and C4b-BP than LA(+)aCL(-) patients (P < 0.05). In some patients, protein S activity (PSact) was also measured and a high correlation was observed between fPS antigen and PSact (r = 0.93, P < 0.001). The data show that the presence of aCL antibodies is associated with a probably acquired deficiency of PS and C4b-BP. On the other hand, in LA patients without a CL antibodies, the fPS deficiency is unrelated to an increase in C4b-BP levels and may be due to abnormal binding of PS to C4b-BP.
采用免疫分析法对73例抗磷脂（aPL）抗体患者检测了总蛋白S（tPS）、游离蛋白S（fPS）和C4b结合蛋白（C4b - BP），以确定先前报道的该组患者PS水平异常是否与狼疮抗凝物（LA）或抗心磷脂（aCL）抗体的存在有关。与正常对照组（n = 44）相比，作者发现45例LA(+)aCL(+)患者的tPS、fPS和C4b - BP显著降低（P < 0.001），8例LA(-)aCL(+)患者的tPS降低（P < 0.001）、fPS和C4b - BP降低（P < 0.01），20例LA(+)aCL(-)患者仅fPS降低（P < 0.05）。LA(+)aCL(+)和LA(-)aCL(+)患者之间的tPS、fPS和C4b - BP水平无差异。相比之下，LA(+)aCL(+)患者的tPS、fPS和C4b - BP值低于LA(+)aCL(-)患者（P < 0.05）。在一些患者中还检测了蛋白S活性（PSact），并观察到fPS抗原与PSact之间高度相关（r = 0.93，P < 0.001）。数据表明，aCL抗体的存在与可能获得性的PS和C4b - BP缺乏有关。另一方面，在无aCL抗体的LA患者中，fPS缺乏与C4b - BP水平升高无关，可能是由于PS与C4b - BP的异常结合所致。
