Summary. The red cell 2,3‐DPG content of unsplenectomized patients with hereditary spherocytosis (HS) was found to be markedly lower than that of other haemolytic disorders with similar reticulocyte values. However, there were no differences in 2,3‐DPG between splenectomized HS patients and healthy subjects. The cause of this decreased 2,3‐DPG in unsplenectomized patients was studied. When heparinized blood both of unsplenectomized and splectomized HS individuals was incubated with excess of glucose, there was a significantly higher splitting of 2,3‐DPG than in the normal subjects. Since the activity of the 2,3‐DPG splitting enzyme, 2,3‐DPGase, was shown to be increased by decreasing pH, the increased 2,3‐DPG splitting during incubation of HS blood could be explained by the increased fall in pH during incubation of the blood, presumably due to increased lactate formation. This assumption was further supported by the finding that 2,3‐DPG splitting both in normal and HS blood was completely inhibited when the pH of blood was raised to 7.8–7.9 and maintained in this range during the incubation. In vitro incubation of blood simulates in some ways in vivo erythrostasis in the spleen and it is suggested that the decreased 2,3‐DPG content found in the fresh red cells of unsplenectomized HS patients is due to increased 2,3‐DPG splitting in these cells during their erythrostasis in the spleen.

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