Electrolyte composition and equilibrium in hemoglobin CC red blood cells.

The red cells of two patients with homozygous hemoglobin C disease (CC) were found to have reduced cation and water content when compared to the red cells of two normal subjects that contained only hemoglobin A (AA). The reduction in cation content was of such a magnitude that the intracellular chloride and hydroxyl (and proton) concentrations were within normal limits despite a measured reduction in the concentration of impermeant negative anions in CC as compared with AA cells of 40 mEq/kg of dry cell solids. The osmotic coefficient of hemoglobin C in CC cells was found to be similar to that observed for hemoglobin A in AA cells. We found no evidence for increased amounts of bound or osmotically abnormal water in CC cells. The reduction in cell cation content in CC cells is mainly due to a reduction in cell K content. The reduced K content is probably related to an increased ouabain- and furosemide-insensitive pathway for K transport in CC cells.