[Glutathione reductase deficiency with membrane defect in hereditary hemolytic anemia]

Glutathione reductase activity and phospholipid metabolism in red cell membranes were determined in a family with hereditary hemolytic anemia. A marked decrease of glutathione reductase activity and stability of reduced glutathione was found in combination with enhanced phospholipid-phosphate metabolism and decreased activity of the membrane-stabilizing enzyme lysolecithin-acyltransferase. In all cases splenectomy beneficially influenced the hemolytic process. Family studies revealed a dominant-autosomal genetic transmission.