The Nonspherocytic Congenital Haemolytic Anaemias
The Nonspherocytic Congenital Haemolytic Anaemias
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非球形细胞先天性溶血性贫血
DOI：
10.1111/j.1365-2141.1972.tb03500.x
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发表时间：
1972
影响因子：
6.5
通讯作者：
A. Grimes
中科院分区：
文献类型：
--
作者：
G. C. Gruchy;A. Grimes
20 GC de Gruchy和A. I.对比两种类型的Grimes特征是自身溶血试验及其通过添加葡萄糖进行的校正的差异。在I型中，他们发现仅全血的自身溶血是正常的，并且添加葡萄糖导致自身溶血减少，尽管少于正常量。在I1型中，仅全血的自身溶血显著增加，并且不因添加葡萄糖而减少。葡萄糖不能防止溶血，这与遗传性球形红细胞增多症的典型发现形成鲜明对比，在遗传性球形红细胞增多症中，葡萄糖通常能防止明显的溶血。此外，通过对葡萄糖消耗的实际测量，他们证明，如果考虑到网织红细胞的高百分比，其中两例I 1型病例中的红细胞消耗葡萄糖的速率仅为正常速率的30%左右。这一重要的观察结果导致了这样的建议：“这些细胞在uitro中的溶解大大增加，可能也在uiuo中，与它们的葡萄糖利用缺陷有关。”非球形红细胞溶血性贫血研究的下一个进展是研究红细胞主要糖酵解途径（Embden-Meyerhof途径）的磷酸盐中间体。因此，Motulsky等（1955）和Prankerd（1957）报道了红细胞2~-二磷酸甘油酸（2，pDPG）增加，其中3例红细胞三磷酸腺苷（ATP）含量低，2例葡萄糖消耗不足。另有几例报告红细胞ATP含量低（de Gruchy et a2，
20 GC de Gruchy and A. I. Grimes feature contrasting the two types was the difference in the autohaemolysis test and in its correction by the addition of glucose. In Type I they found that autohaemolysis of whole blood alone was normal and that the addition of glucose caused the autohaemolysis to be diminished although by less than the normal amount. In Type I1 the autohaemolysis of the whole blood alone was markedly increased and was not diminished by the addition of glucose. This failure of glucose to prevent haemolysis was in marked contrast to the typical finding in hereditary spherocytosis in which glucose usually prevented significant haemolysis. Furthermore, by actual measurements of glucose consumption they demonstrated that the red cells in two of the Type I1 cases consumed glucose at only about 30% of the normal rate, if allowance was made for the high percentage of reticulocytes present. This important observation led to the suggestion that ‘the greatly increased lysis of these cells in uitro, and probably also in uiuo, is related to their defective glucose utilization.’The next development in the investigation of nonspherocytic haemolytic anaemia was the study of the phosphate intermediates of the main glycolytic pathway of the red cell, the Embden-Meyerhof pathway. Thus an increase in the red cell 2~-diphosphoglycerate (2, pDPG) was reported by Motulsky et al (1955) and Prankerd (1957) reported three cases with low red cell adenosine triphosphate (ATP) content, with deficient glucose consumption in two. Several more cases were reported with a low red cell ATP content (de Gruchy et a2,