Life-Span and Organ Sequestration of the Red Cells in Pyruvate Kinase Deficiency

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Abstract
To define the role of the spleen in the hemolytic anemia associated with erythrocyte pyruvate kinase (PK) deficiency, removal rates and patterns or organ sequestration of 51Cr-labeled PK erythrocytes were studied. Recipients were the PK patients themselves, normal subjects and persons splenectomized for diverse causes. In one study the 51Cr was attached to "young" and "old" populations of PK erythrocytes before their injection; in another the "young" population was differentially labeled with glycine-2–14C. The results show that the PK-deficient cell is heterogeneously affected by whatever mechanisms induce its destruction. Therefore, a population of "young" PK cells contains more seriously damaged cells than a population of older cells, the latter living to an older age because of their improved initial outlook. Younger cells, if they pass through the spleen, are subsequently destroyed in the liver. Removal of the spleen improves the survival of the younger cells, for they are then destroyed much more slowly in the liver. Splenectomy is indicated in the hemolytic anemia associated with pyruvate kinase deficiency.
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