Rosse W F, Adams J P
Prog Clin Biol Res. 1979;30:457-61.
Paroxysmal nocturnal hemoglobinuria (PNH) cells are characterized by an abnormal membrane which interacts abnormally with normal serum complement. These abnormal reactions occur at two levels of the sequence: 1) All abnormal PNH cells fix more C3 than normal cells for a given activation of complement; 2) PNH III cells are more readily lysed by a given number of assembled terminal complexes. The nature of the lesion yielding these abnormal reactions is not known.
阵发性夜间血红蛋白尿(PNH)细胞的特征是其膜异常,与正常血清补体发生异常相互作用。这些异常反应发生在补体激活序列的两个层面:1)对于给定的补体激活,所有异常PNH细胞比正常细胞结合更多的C3;2)一定数量的组装终末复合物更容易裂解PNH III细胞。导致这些异常反应的病变性质尚不清楚。