Abstract
The rheological properties of enzyme-deficient erythrocytes differ significantly. In glucosephosphate isomerase deficiency young as well as whole erythrocyte populations show a markedly increased rigidity and an abnormally strong attachment of hemoglobin to the inner surface of isolated membranes. Glucose-6-phosphate dehydrogenase-deficient erythrocytes are more flexible than normal erythrocytes. Even after acetylphenyliiydrazin-induced Heinz body formation these cells are less rigid than normal erythrocytes incubated under identical conditions. The flexibility of pyruvate kinase deficient erythrocytes and reticulocytes is normal in a favourable surrounding. During incubation at low pH and low glucose concentrations especially the reticulocytes become highly rigid. The rheological properties of the enzyme-deficient erythrocytes explain why splenectomy improves the red cell lifespan in glucosephosphate isomerase and pyruvate kinase deficiency, out not in glucose-6-phosphate dehydrogenase deficiency.
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Schröter, W., Tillmann, W. & Gahr, M. MEMBRANE FLEXIBILITY OF ENZYME-DEFICIENT ERYTHROCYTES. Pediatr Res 12, 71 (1978). https://doi.org/10.1203/00006450-197801000-00067
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DOI: https://doi.org/10.1203/00006450-197801000-00067
