Red cell life span in sickle cell hemoglobin C disease with a note about sickle cell hemoglobin Oarab

Abstract

Red cell survival was measured in 10 subjects with SC disease and one with SO Arab (a2ß2121 glu?lys) disease using both DF32P and51Cr as tags. Red cell volume was slightly reduced in most patients (87% ± 20% of predicted normal). In 9 SC patients, mean red cell life (DF32P) was 28.9 ± 4.0 days. For one SC subject it was significantly longer (47.9 days), as it was for the one with SO Arab. The SO Arab subject had irreversibly sickled cells in the peripheral blood, whereas those with SC had few (<1/1000 red cells) or none. The SO Arab hemolysate gelled at a hemoglobin concentration (16.2 g/100 ml) near that for sickle cell anemia hemolysates (15.9 ± 1.0 g/100 ml; n= 8) but significantly lower than that for SC hemolysates (21.6 ± 1.9 g/100 ml; n= n=5). It seems likely that properties of SC red cells other than their relative ease of sickling contribute significantly to their rate of hemolysis.