Hereditary Hemolytic Anemia with Hexokinase Deficiency

Abstract

IT is now clear that hereditary hemolytic anemias not associated with hemoglobinopathy or with the thalassemia syndromes are frequently due to inherited deficiencies in one or another of the enzymes upon which the erythrocyte is dependent to meet its energy needs. The non-nucleated, relatively metabolically impoverished adult human red cell has limited resources and places major dependence upon energy derived from the conversion of glucose to lactate – chiefly via the anaerobic Embden–Meyerhof pathway, but to a lesser but still important extent by way of the oxidative hexose monophosphate shunt. In the former diphosphopyridine nucleotide (NAD) is cycled to its . . .