Hemolytic Anemia Associated with Low Erythrocyte ATP

Studies have been carried out on erythrocytes of a young girl with a severe hemolytic anemia. Biochemically, these cells were characterized by a very high glycolytic rate, a low ATP level, an elevated ADP:ATP ratio, and a markedly increased rate of catabolism of adenine nucleotides to hypoxanthine during incubation in vitro.

Specific enzyme assays of the patient’s cells revealed normal or elevated values for glucose-6-phosphate dehydrogenase, pyruvate kinase, glutathione reductase, enolase, adenosine triphosphatase, and adenine phosphoribosyl transferase.

In in vitro incubation studies, addition of adenine to the blood brought about an increase in the ATP level and in total adenine nucleotides. These data suggest that the low ATP level in the patient’s blood was due to increased catabolism of adenine nucleotides, probably as a consequence of markedly increased utilization of ATP by the cell.