RED-CELL ENZYMES AND CO-ENZYMES IN NON-SPHEROCYTIC CONGENITAL HAEMOLYTIC ANAEMIAS.

Abstract

The non‐spherocytic congenital haemolytic anaemias were classified on haematological grounds by Selwyn and Dacie (1954) into two main types—Type I and Type II. It now seems that Type I cases probably form a heterogeneous group while the Type II cases are probably a homogeneous group (de Gruchy, Santamaria, Parsons and Crawford, 1960). Robinson, Loder and de Gruchy (1961) presented evidence of impairment of cell metabolism in both Type I and Type II cases and suggested that in Type II cases there was an enzymatic defect in glycolysis beyond the stage of 2,3‐diphosphoglycerate (2,3‐DPG) synthesis. This was confirmed by the demonstration by Tanaka, Valentine and Miwa (1962) of a pyruvate kinase (PK) deficiency in Type II cases.This paper reports observations in a number of patients with Type I or Type II non‐spherocytic haemolytic anaemia on (a) the activities of the enzymes of the Embden–Meyerhof glycolytic pathway, (b) the activities of the enzymes glucose‐6‐phosphate dehydrogenase and glutathione reductase, (c) the values of the co‐enzymes diphosphopyridine nucleotide (DPN) and reduced diphosphopyridine nucleotide (DPNH) and (d) the values of reduced glutathione. The significance of the findings is discussed together with their relevance to the classification of these anaemias.