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Acute Hemolytic Anemia Associated With Erythrocyte Glutathione-Peroxidase Deficiency

Martin Steinberg, MD; Mark J. Brauer, MD; Thomas F. Necheles, MD
Arch Intern Med. 1970;125(2):302-303. doi:10.1001/archinte.1970.00310020108013.
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In 1968, Necheles and co-workers described the first instances of hemolytic anemia associated with deficiency of erythrocyte glutathione peroxidase (GSH-Px).1,2

Abnormalities of the pentose phosphate shunt leading to hemolytic anemia were first noted in 1956 when Carson and associates showed that erythrocytes of individuals susceptible to the hemolytic effects of primaquine were deficient in the enzyme glucose-6-phosphate dehydrogenase (G6PD).3 Since then, deficiencies of the other shunt enzymes, glutathione reductase 4 and 6-phosphogluconate dehydrogenase 5 and of glutathione6 have been described. These enzyme deficiencies may be associated at times with hemolytic anemia, which may occur spontaneously, or be precipitated by administration of oxidant drugs. In some instances, infection has been associated with the development of hemolytic anemia in individuals deficient in G6PD.7,8

Previous reports include the presence of hemolytic disease in neonates with heterozygous GSH-Px deficiency 1 and homozygous deficiency in an adult who had a hemolytic reaction after a transfusion


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