Abstract
A six year old girl suffering from severe nonspherocytic hemolytic anemia which was characterized by Stomatocytosis, macrocytosis, and autohemolysis type I was found to have erythrocytes with reduced potassium (16 mval/l) and elevated sodium (95 mval/1; concentrations. Glykolysis, ATP formation and ATP utilization of the cells were markedly increased. Active sodium outflux and potassium influx were increased ten- and six fold, respectively. Inhibition of the cation pump by ouabain decreased the active cation fluxes for about 60 %. Passive sodium and potassium fluxes were likewise markedly increased. Normal red cells reconstituted with intracellular cation concentrations which were similar to the concentrations of the abnormal cells showed only a slight increase of active cation fluxes. The results suggest a defect of passive cation permeability of the abnormal cells.
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