Extreme Hemolysis and Red-Cell Distortion in Erythrocyte Pyruvate Kinase Deficiency

Abstract

IN a preceding paper¹ the clinical, morphologic, enzymatic and erythrokinetic abnormalities attending the violent chronic hemolytic anemia of M.P., a child with homozygous pyruvate kinase deficiency, were described. The following discussion deals with certain disturbances of the metabolism of his erythrocytes that appear to contribute to the mechanisms of hemolysis in this disorder. The data are also of broader interest because they may afford additional insight into the general mechanisms of the death of erythrocytes in man.Materials and MethodsIn vitro studies of the patient's erythrocyte potassium flux, potassium and water contents, glucose consumption and adenosine triphosphate (ATP) stability . . .