Gigli I, Schothorst A A, Soter N A, Pathak M A
J Clin Invest. 1980 Sep;66(3):517-22. doi: 10.1172/JCI109883.
The complement system was analysed in 14 asymptomatic patients with erythropoietic protoporphyria. In the majority of the sera studied the levels of complement components C1, C4, C2, and C3 were within the normal range. Upon ultraviolet light (330--460 nm) irradiation of the serum samples in vitro, a marked decrease in total hemolytic activity accompanied by reduction of C1, C4, C2, and C3 levels was observed. The loss of total hemolytic activity can be directly correlated with the levels of protoporphyrin (PP) and similar changes can be obtained in normal serum upon addition of PP followedf by ultraviolet light irradiation. It is postulated that after irradiation the excited PP develops the capacity to activate the complement sequence with the production of cleavage products, which may contribute to the skin changes observed in these patients upon sun exposure.
对14例无症状红细胞生成性原卟啉病患者的补体系统进行了分析。在大多数研究的血清中，补体成分C1、C4、C2和C3水平在正常范围内。在体外对血清样本进行紫外线（330 - 460nm）照射后，观察到总溶血活性显著降低，同时C1、C4、C2和C3水平降低。总溶血活性的丧失与原卟啉（PP）水平直接相关，在正常血清中加入PP后再进行紫外线照射也可获得类似变化。据推测，照射后激发态的PP产生了激活补体序列并产生裂解产物的能力，这可能导致这些患者在阳光照射后出现皮肤变化。
