Hillmen P, Hows J M, Luzzatto L
Department of Haematology, Royal Postgraduate Medical School, London.
Br J Haematol. 1992 Mar;80(3):399-405. doi: 10.1111/j.1365-2141.1992.tb08151.x.
We have studied three glycosylphosphatidylinositol (GPI) linked proteins on the erythrocytes of 14 patients with paroxysmal nocturnal haemoglobinuria (PNH). The pattern observed was bimodal in 12 of the patients and trimodal in two. Ten patients had a red cell population with normal CD59 antigen (membrane inhibitor of reactive lysis, MIRL), decay accelerating factor (DAF or CD55) and lymphocyte function-associated antigen (LFA-3 or CD58) and a second abnormal PNH population with absent CD59 antigen, DAF and LFA-3. The other two patients with a bimodal pattern had a red cell population with normal CD59 antigen, DAF and LFA-3 and an abnormal population with reduced, but not absent, CD59 antigen and DAF. The LFA-3 on the abnormal red cells in these two patients appeared to be only slightly reduced. The two patients with a trimodal pattern had a normal population, a population with reduced, not absent, CD59 antigen and DAF, and a population with complete absence of CD59 antigen, DAF and LFA-3. The accuracy of the Ham test in estimating the proportion of red cells with the PNH defect in the two types of PNH was assessed. The case of one patient who appeared to be 'rescued' from severe aplastic anaemia by the development of PNH is described.
我们研究了14例阵发性夜间血红蛋白尿（PNH）患者红细胞上的三种糖基磷脂酰肌醇（GPI）连接蛋白。观察到的模式在12例患者中为双峰模式，在2例患者中为三峰模式。10例患者有一个红细胞群体，其CD59抗原（反应性溶解膜抑制剂，MIRL）、衰变加速因子（DAF或CD55）和淋巴细胞功能相关抗原（LFA-3或CD58）正常，另一个异常的PNH群体则缺乏CD59抗原、DAF和LFA-3。另外两名具有双峰模式的患者有一个红细胞群体，其CD59抗原、DAF和LFA-3正常，另一个异常群体的CD59抗原和DAF减少但并非缺失。这两名患者异常红细胞上的LFA-3似乎仅略有减少。两名具有三峰模式的患者有一个正常群体、一个CD59抗原和DAF减少但并非缺失的群体，以及一个完全缺乏CD59抗原、DAF和LFA-3的群体。评估了Ham试验在估计两种类型PNH中具有PNH缺陷的红细胞比例方面的准确性。描述了一名患者的病例，该患者似乎因PNH的发展而从严重再生障碍性贫血中“获救”。
