Splenectomy For Hereditary Spherocytosis Research Articles

124. A case of splenectomy in hereditary spherocytosis of child

124. A case of splenectomy in hereditary spherocytosis of child

Abnormalities of Carbohydrate Metabolism of Red Cells in Hereditary Spherocytosis

By a technique for investigating glycolysis of red cells, further insight was gained into the nature of the disturbances in metabolism associated with hereditary spherocytosis. A defect in intracellular glycolysis was found in cells from patients with hereditary spherocytosis. The abnormalities persist after splenectomy. The abnormalities in carbohydrate metabolism are correlated with the clinical features of the disease. The susceptibility of red cells to hemolysis may be associated with an inadequate supply or utilization of energy within the cells. Spherocytes possessing this abnormality when trapped within the spleen may be faced with relative starvation, making them more susceptible to hemolysis. When the spleen is removed, such cells may survive normally because of more ready access to the plasma constituents, despite the continued presence of the metabolic defects in glycolysis. Thus the beneficial effects of splenectomy in hereditary spherocytosis may be more understandable.