Z Gesamte Inn Med. 1976 May 1;31(9):257-61.
Hereditary non-spherocytary haemolytic anaemias have their cause in enzymopathies of the pentose phosphate cycle and the glycolysis of the erythrocytes. The 11 known enzyme defects of the erythrocytary glycolysis in consequence of the reduced preparation of adenosine triphosphatase condition a deficient stability of the membrane of the erythrocytes. Therefore, the increased autohaemolysis in normal osmotic resistance is a reference to these forms of anaemia, which are particularly to be differentiated from hereditary sperocytoses. In Middle Europe the deficiency of pyruvate kinase plays the greatest part among the otherwise rarely diagnosed enzymopenic haemolytic anaemias.
遗传性非球形红细胞溶血性贫血是由红细胞磷酸戊糖循环和糖酵解中的酶病引起的。红细胞糖酵解中已知的11种酶缺陷会导致三磷酸腺苷酶的合成减少，从而导致红细胞膜稳定性不足。因此，在正常渗透压抵抗力下自身溶血增加是这些贫血形式的一个参考指标，尤其需要与遗传性球形红细胞增多症相鉴别。在中欧，丙酮酸激酶缺乏在其他罕见诊断的酶缺乏性溶血性贫血中起最大作用。
