Zarkowsky H S, Mohandas N, Speaker C B, Shohet S B
Br J Haematol. 1975 Apr;29(4):537-43. doi: 10.1111/j.1365-2141.1975.tb02740.x.
Microspherocytes, measuring 2-3 mum in diameter, and cells with blunted projections or triangular in shape characterized the erythrocoyte morphology in three children with congenital haemolytic anaemia. Since the erythrocyte morphology resembled that associated with thermal injury, heat-induced changes in erythrocyte morphology and membrane composition were studied. Erythrocytes developed filaments and spheroid bodies which fragmented, resulting in microspherocyte transformation. Normal cells required exposure to 49 degrees C, whereas the patients' cells fragmented at 45 degrees C. Fragmentation was also observed during incubation of patients' cells at 37 degrees C for 17h. The heat-induced transformation of the patients' cells was associated with an increase in the membrane cholesterol:phospholipid and cholesterol:protein ratios. The phospholipid:protein ratio was unchanged. This suggests that fragmentation produces a selective loss of membrane components. Splenectomy ameliorated the haemolytic process. We propose that the patients' red-cell morphology is the result of in vivo fragmentation, and that the spleen is the major site of microspherocyte and poikilocyte destruction.
在三名先天性溶血性贫血患儿中，红细胞形态以直径为2 - 3微米的小球形红细胞以及具有钝性突起或呈三角形的细胞为特征。由于红细胞形态类似于热损伤相关的形态，因此对热诱导的红细胞形态和膜成分变化进行了研究。红细胞形成了细丝和球状体，这些细丝和球状体随后破碎，导致小球形红细胞转化。正常细胞需要暴露在49摄氏度下，而患者的细胞在45摄氏度时就会破碎。在将患者的细胞于37摄氏度孵育17小时的过程中也观察到了破碎现象。患者细胞的热诱导转化与膜胆固醇:磷脂和胆固醇:蛋白质比率的增加有关。磷脂:蛋白质比率未发生变化。这表明破碎导致了膜成分的选择性丢失。脾切除术改善了溶血过程。我们认为，患者红细胞形态是体内破碎的结果，并且脾脏是小球形红细胞和异形红细胞破坏的主要部位。
