Devine D V, Siegel R S, Rosse W F
J Clin Invest. 1987 Jan;79(1):131-7. doi: 10.1172/JCI112773.
The blood cells of patients with paroxysmal nocturnal hemoglobinuria (PNH) have abnormal interactions with complement. The activity of the alternative pathway C3 convertase on the platelets of 9 out of 19 patients with PNH was elevated. 10 patients had C3 convertase activity within the normal range even though 80-95% of their platelets lacked the complement regulatory protein decay accelerating factor (DAF) that is absent from the affected blood cells in PNH. PNH and normal platelets released factor H when C3 was bound to their surfaces. This may account for the apparent regulation of C3 convertase activity on platelets that lack DAF. The abnormal uptake of the membrane attack complex of complement by PNH III erythrocytes was not seen in PNH platelets. 111Indium-labeled platelet survival times were normal in five of eight patients, which suggests that the lack of the membrane attack complex defect results in normal platelet survival in PNH.
阵发性夜间血红蛋白尿（PNH）患者的血细胞与补体存在异常相互作用。19例PNH患者中有9例血小板上替代途径C3转化酶的活性升高。尽管10例患者80% - 95%的血小板缺乏补体调节蛋白衰变加速因子（DAF），而PNH患者受影响的血细胞中不存在该因子，但这10例患者的C3转化酶活性仍在正常范围内。当C3结合到PNH和正常血小板表面时，二者均释放因子H。这可能解释了在缺乏DAF的血小板上C3转化酶活性的明显调节。PNH III型红细胞对补体膜攻击复合物的异常摄取在PNH血小板中未观察到。8例患者中有5例的铟 - 111标记血小板存活时间正常，这表明膜攻击复合物缺陷的缺乏导致PNH患者血小板存活正常。
