Stern M, Rosse W F
Blood. 1979 May;53(5):928-34.
The granulocytes in paroxysmal nocturnal hemoglobinuria (PNH) are defective, and the defect is similar to that previously described for the PNH erythrocyte. Using anti-I antibody to activate complement and 51Cr release to detect cell lysis, we found two populations of granulocytes that differed in their susceptibility to lysis by complement in 5 of 6 patients. A proportion of the cells were lysed by one-fifteenth to one-twentieth the amount of complement required to lyse normal cells; the remainder of the granulocytes appeared to be normal in their susceptibility to the lytic action of complement. The binding of the third component of complement (C3) to PNH granulocytes was at least twice that bound to normal cells, even though the binding of antibody was the same for normal and PNH cells. This suggests that the binding of C3 and probably the efficiency of the terminal steps of complement lysis are increased in the abnormal PHN granulocyte. These defects affect only a portion of the granulocytes, thus suggesting that the disorder is a clonal stem cell abnormality.
阵发性睡眠性血红蛋白尿（PNH）中的粒细胞存在缺陷，且该缺陷与先前描述的PNH红细胞的缺陷相似。使用抗I抗体激活补体并通过51Cr释放检测细胞裂解，我们在6例患者中的5例中发现了两类对补体裂解敏感性不同的粒细胞。一部分细胞被裂解所需的补体量是裂解正常细胞所需补体量的十五分之一到二十分之一；其余粒细胞对补体的裂解作用的敏感性似乎正常。补体第三成分（C3）与PNH粒细胞的结合至少是与正常细胞结合量的两倍，尽管正常细胞和PNH细胞的抗体结合量相同。这表明异常的PNH粒细胞中C3的结合以及补体裂解终末步骤的效率可能有所提高。这些缺陷仅影响一部分粒细胞，因此提示该疾病是一种克隆性干细胞异常。
