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E Beutler, D Dunning, IB Dabe and L Forman
Research Institute of Scripps Clinic, Department of Basic and Clinical
Research, La Jolla, CA 92037.
A patient with unexplained erythrocyte glutathione-S-transferase (GST)
deficiency has been detected among 513 unrelated persons with hemolytic
anemia. An otherwise healthy adult male, the deficient individual had a
mild hemolytic anemia with splenomegaly, indirect hyperbilirubinemia, and
cholelithiasis. Because he was adopted and childless, the hereditary nature
of the defect could not be established. The residual enzyme activity was
only about 15% of mean normal. Depletion of glutathione (GSH) from the
cells by 1-chloro-2,4-dinitrobenzene (CDNB), a substrate for GST, was
somewhat decreased in the red cells from the patient, suggesting that a
functional defect existed. The kinetic properties of the residual enzyme
and the ratio of activity to antigenicity were normal. Modest decreases in
leukocyte and platelet GST activities were documented. Although a
cause-and-effect relationship between the GST deficiency and hemolysis may
exist, this cannot be proven in the absence of affected family members.
This article has been cited by other articles:
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| Copyright © 1988 by American Society of Hematology Online ISSN: 1528-0020 | |||||||||
