Incubation Hemolysis and Red Cell Metabolism in Acanthocytosis*

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Abstract
The red cells of two males, aged 6 and 8, having acanthocytosis and a-betalipoproteinemia were studied. Red cells in acanthocytosis can be segregated on the basis of their age by centrifugal fractionation. The younger cells show minimal or no morphologic distortion; the degree of cell deformity increases with cell maturation or aging or both. Glycolysis, phosphogluconate oxidation, and pentose phosphate metabolism in acanthocytes are characteristic of a population slightly enriched with young cells. The capacity of acanthocytes to utilize these pathways for reduced glutathione and adenosine triphosphate maintenance and for methemoglobin reduction is normal. Red cells in acanthocytosis become permeable to hemoglobin more rapidly than normal cells during 48 hours incubation at 37[degree]C and at 4[degree]C. Inhibition of the lysis can be produced a) by certain conditions of incubation leading to better preservation of intracellular adenosine triphosphate, b) by the addition of normal serum, and c) by heparin. Accentuation of hemoglobin permeability occurs in ethylenediaminetetraacetate, possibly due to chelation of Mg++. The extensive lysis of acanthocytosis blood, collected into ethylenediaminetetraacetate and kept at room temperature for 48 to 72 hours, may serve as a convenient screening test for this uncommon disorder. The in vivo life span of the red cells is probably slightly shortened, but this accelerated destruction may be intermittent. No abnormality in morphology or autohemolytic behavior was found in the red cells from the parents and brother of one patient.