Nilsson I M, Sundqvist S B, Ljung R, Holmberg L, Freiburghaus C, Björlin G
Scand J Haematol. 1983 May;30(5):458-64. doi: 10.1111/j.1600-0609.1983.tb02534.x.
A 39-year-old patient, suffering from severe haemophilia B and antibodies against factor IX, has twice been treated with extracorporeal protein A-Sepharose adsorption followed by conventional substitution therapy in combination with immunosuppression (cyclophosphamide). On both occasions, separated by a 2-year interval, the same procedure was followed except that, on the second, administration of i.v. immunoglobulin (Gammonativ. KabiVitrum) was added. Within a week of the first treatment the patient developed a 15-fold increase in the antibody titre. Following the second treatment described here, no secondary antibody response could be detected, and after a further 12 weeks only traces of antibodies are demonstrable. It seems that antibody synthesis was suppressed by the i.v. immunoglobulin. No evidence was found to demonstrate that the effect was due either to a non-specific suppression of the immune and reticuloendothelial systems or to the action of interfering antibodies. It has not yet been established whether or not the protein A-Sepharose adsorption technique, or the immunosuppressive treatment, contributed in any way to the result. The observations suggest a new approach to the treatment of haemophiliacs with antibodies of the high-responding type.
一名39岁的患者患有严重的乙型血友病且体内存在抗凝血因子IX抗体,曾两次接受体外蛋白A-琼脂糖吸附治疗,随后进行传统替代疗法并联合免疫抑制(环磷酰胺)。两次治疗间隔两年,治疗过程相同,只是在第二次治疗时添加了静脉注射免疫球蛋白(Gammonativ. KabiVitrum)。第一次治疗后一周内,患者抗体滴度增加了15倍。在此处描述的第二次治疗后,未检测到二次抗体反应,再过12周后仅可检测到微量抗体。似乎静脉注射免疫球蛋白抑制了抗体合成。未发现证据表明该效果是由于免疫和网状内皮系统的非特异性抑制或干扰抗体的作用。蛋白A-琼脂糖吸附技术或免疫抑制治疗是否对结果有任何贡献尚未确定。这些观察结果提示了一种治疗高反应型抗体血友病患者的新方法。