ERYTHROCYTE CARBOHYDRATE METABOLISM IN HEREDITARY SPHEROCYTOSIS*

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Abstract
A profile of carbohydrate metabolism in erythrocytes from patients with hereditary spherocytosis (HS) was obtained by incubating HS erythrocytes at 37[degree]C and measuring changes in the concentration of glucose, lactate, pyruvate, ketose and triose phosphates, 2,3-diphosphoglycerate, adenosine triphosphate (ATP), and pentose phosphates, the latter analyzed to give values for purine-bound and purine-free fractions. Similar studies were made after the addition of methylene blue or iodoacetate. Although erythrocytes drawn from splenectomized patients with HS utilized glucose at a normal rate, and appeared to possess an intact pentose phosphate pathway, they showed some difficulty in maintaining a normal level of purine-bound pentose, but not ATP, in the presence of methylene blue. Several other aberrations were noted which proved statistically significant, but the relation of these to a fundamental defect in the HS erythrocyte is unclear.