Verroust F, Adam C, Kourilsky O, Allain J P, Verroust P
J Clin Lab Immunol. 1981 Sep;6(2):127-30.
Immune complexes and complement levels were assayed in sera from a group of 69 hemophilic children. Using the Raji cell radioimmune assay and the C1q binding assay, abnormally high levels of circulating immune complexes were rarely found in the group of hemophiliacs which did not differ statistically from the control population. These results do not exclude the presence of low and transient levels of immune complexes in the circulation, but indicate that hemophiliacs are not exposed to increased immune complex loads similar to those found in immune complex diseases. By contrast frequent abnormalities of the complement system were found. Complement levels were elevated in a large percentage of patients, reaching statistical significance for C3, C4, C5, Factor B and Properdin. Levels of the C3 breakdown product C3d were significantly raised suggesting intravascular complement activation. The significance of these abnormalities is discussed in relation to perfusion of Factor VIII preparations.
对一组69名血友病儿童的血清进行了免疫复合物和补体水平检测。采用拉吉细胞放射免疫测定法和C1q结合测定法，在血友病患者组中很少发现循环免疫复合物水平异常升高，且与对照组相比无统计学差异。这些结果并不排除循环中存在低水平和短暂的免疫复合物，但表明血友病患者并未像免疫复合物疾病患者那样暴露于增加的免疫复合物负荷中。相比之下，发现补体系统频繁出现异常。很大一部分患者的补体水平升高，C3、C4、C5、B因子和备解素达到统计学显著水平。C3裂解产物C3d的水平显著升高，提示血管内补体激活。结合因子VIII制剂的灌注情况讨论了这些异常的意义。
