Hauptmann G, Petitjean F, Lang J M, Oberling F
Clin Exp Immunol. 1979 Sep;37(3):523-31.
A patient with an extensive lymphosarcoma of the spleen without involvement of other lymphoid organs and hypogammaglobulinaemia showed the characteristic complement profile of an acquired C1-inhibitor deficiency. Both functional and immunochemical studies revealed extremely low levels of the inhibitor of C1-esterase. Correction of the low levels of early acting complement components and of the low C1-inhibitor level followed the splenectomy. In vitro tests showed that lymphosarcoma tissue pieces or cells were able to interact with complement, resulting in a depletion of the haemolytic activity. These findings provide evidence that tumour cells were responsible for the abnormalities of the complement system.
一名患有广泛脾脏淋巴肉瘤且无其他淋巴器官受累及低丙种球蛋白血症的患者，表现出获得性C1抑制物缺乏的特征性补体谱。功能和免疫化学研究均显示C1酯酶抑制物水平极低。脾切除术后，早期起作用的补体成分水平降低及C1抑制物水平降低的情况得到纠正。体外试验表明，淋巴肉瘤组织块或细胞能够与补体相互作用，导致溶血活性耗竭。这些发现证明肿瘤细胞是补体系统异常的原因。
