Jordon R E, McDuffie F C, Good R A, Day N K
Clin Exp Immunol. 1974 Nov;18(3):407-15.
A patient with diffuse normolipaemic plane xanthomatosis, a benign monoclonal gammopathy, and a low total serum complement level is described. Functional haemolytic assays revealed decreased levels of C1, C4 and C2 components and elevated levels of the two terminal components C8 and C9. Radial immunodiffusion demonstrated low levels of C1q and C4 and a high level of C3. Both functional and immunochemical studies revealed extremely low levels of the inhibitor of C1 esterase. Anticomplementary activity was not present in the patient's serum. Despite low concentrations of the early reacting components of the classic complement pathway, the alternate pathway seemed to be intact. It was concluded that the patient had the recently delineated syndrome of acquired deficiency of the C1-esterase inhibitor.
本文描述了一名患有弥漫性正常血脂性扁平黄瘤病、良性单克隆丙种球蛋白病且血清总补体水平较低的患者。功能性溶血试验显示C1、C4和C2成分水平降低，而两个终末成分C8和C9水平升高。放射免疫扩散法显示C1q和C4水平较低，C3水平较高。功能性和免疫化学研究均显示C1酯酶抑制剂水平极低。患者血清中不存在抗补体活性。尽管经典补体途径早期反应成分浓度较低，但替代途径似乎完好无损。结论是该患者患有最近明确的获得性C1酯酶抑制剂缺乏综合征。
