ABNORMALITIES OF CARBOHYDRATE METABOLISM OF RED CELLS IN HEREDITARY SPHEROCYTOSIS 12

Abstract

Carbo-hydrate metabolism was studied in the red cells from 18 patients with hereditary spherocytosis by using P32-labeled orthophosphate to measure the rate of incorporation of P32 into the various organic phosphate esters. The phosphate compounds were identified by 2-dimensional paper chromatography. A defect in intracellular glycolysis involving a smaller flux of P32 into adenosinetriphosphate (ATP) and 2,3-DPG with a concurrent increase in the flux into orthophosphate was observed in the red cells from each patient studied. The turnover of P32 of ATP and 2,3-DPG in the stromal fraction tended to be lower than normally found. These abnormalities persisted after splen-ectomy and in 10 cases were largely restored to normal on incubation of the red cells with adenosine. Lack of corrective effect of adenosine in 8 cases suggested that the group of patients thought to have hereditary spherocytosis might not be homogeneous.