BIOCHEMICAL AND GENETIC ASPECTS OF PRIMAQUINE-SENSITIVE HEMOLYTIC ANEMIA

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Abstract
In view of the pattern of enzyme abnormalities, the authors pose the question "Is it possible for one gene to affect directly or indirectly a variation in the production of more than one enzyme?" They conclude that the in vitro instability of reduced glutathione of erythrocytes during incubation with acetylphenylhydrazine is not identical with the phenotype, in vivo hemolysis. Final determination of the mode of genetic transmission of primaquine-sensitive hemolytic anemia must await more definitive genetic and biochemical data. This study emphasized the difficulty of choosing the proper phenotype in order to determine the mode of genetic transfer of inherited disease in man. The clinical significance of this disorder lies in that this type of acute intravascular hemolysis is frequently an iatrogenically produced disease.