Autohemolysis and other changes resulting from the incubation in vitro of red cells from patients with congenital hemolytic anemia.
Autohemolysis and other changes resulting from the incubation in vitro of red cells from patients with congenital hemolytic anemia.
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先天性溶血性贫血患者红细胞体外培养引起的自溶和其他变化。
作者：
J. Selwyn;J. Dacie
观察了37 ℃血清孵育红细胞体积、渗透脆性和阳离子含量的变化。24和48小时。结果表明，自发性自身溶血不是由于细胞的进行性肿胀，而可能是由于细胞膜的退行性变化。
在孵育过程中，由于钠和水的增加，正常的红细胞在最初的24小时内体积增加;细胞失去钾，但速度低于钠的获得。在培养的第二个24小时内，钾的损失超过钠的增加，细胞缩小到接近其原始体积。如果在整个48小时的孵育过程中存在葡萄糖，则这些阳离子变化和自身溶血大大减少。
还研究了几种不同类型的先天性溶血性贫血的红细胞;观察到与正常模式的重要偏差。遗传性球形红细胞增多症的自身溶血率、渗透脆性增加率和钾丢失率均高于正常值。在孵育过程中葡萄糖的持续存在明显延缓了这些变化。
在遗传性椭圆形红细胞增多症中，红细胞在孵育时表现正常。在一例椭圆形红细胞增多症合并溶血性贫血的病例中，自身溶血是正常的，但钾丢失增加。在另一个溶血性贫血和渗透脆性增加的患者中，自身溶血大大增加。在所有这些椭圆形红细胞增多症的病例中，葡萄糖适度降低了自身溶血，但未达到正常程度。
本文报告4例先天性非球形红细胞溶血性贫血。在2例患者（1型）中，自身溶血、渗透脆性和孵育时的阳离子变化均正常;葡萄糖对脆性和阳离子变化有正常影响，但仅轻微降低自身溶血。在另外两名患者（2型）中，自身溶血、渗透脆性增加和钾丢失显著增加。葡萄糖并不阻碍这些变化，并且发现细胞不能以正常速率利用葡萄糖。
Observations were made on the changes in volume, osmotic fragility, and cation contents of red cells incubated in serum at 37 C. for 24 and 48 hours. The results show that spontaneous autohemolysis is not due to progressive swelling of the cells, but is probably due to degenerative changes in the cell membranes.
On incubation, normal red cells increase in volume during the first 24 hours due to a gain in sodium and water; the cells lose potassium but at a slower rate than they gain sodium. During the second 24 hours of incubation the loss in potassium exceeds the gain in sodium and the cells shrink to near their original volume. These cation changes and the autohemolysis are greatly reduced if glucose is present throughout the 48 hours of incubation.
Red cells from several different types of congenital hemolytic anemia were also studied; important deviations from the normal pattern were observed. In hereditary spherocytosis the rates of autohemolysis, of increase in osmotic fragility, and of potassium loss are greater than normal. The continued presence of glucose during incubation markedly retarded these changes.
In hereditary elliptocytosis trait the red cells behaved normally on incubation. In one case of elliptocytosis with hemolytic anemia, autohemolysis was normal but there was an increased potassium loss. In another patient with hemolytic anemia and increased osmotic fragility autohemolysis was greatly increased. In all these cases of elliptocytosis, glucose reduced the autohemolysis moderately but not to a normal degree.
Four cases of congenital nonspherocytic hemolytic anemia were studied. In two patients (type 1) autohemolysis, osmotic fragility and cation changes on incubation were normal; glucose had a normal effect on the fragility and cation changes, but only slightly reduced the autohemolysis. In the two other patients (type 2) autohemolysis, increase in osmotic fragility, and loss of potassium were markedly increased. Glucose did not retard any of these changes and it was found that the cells were unable to utilize glucose at the normal rate.