Hereditary Hemolytic Anemia Associated With Abnormal Membrane Lipid. II. Ion Permeability and Transport Abnormalities

A familial nonspherocytic hemolytic anemia with altered red cell membrane lipids has been described. The membrane lipid lesion is also accompanied by excessive cation permeability and ouabain-sensitive pumping, which is in turn associated with excessive glycolytic energy diverted to the pump. This permeability defect and its demands for glycolytic energy are thought to contribute significantly to the early demise of the red cell in this disorder.