Abstract

The effects of carbamylation and frozen storage on the autologous 51Cr survival and metabolic features of sickle erythrocytes (S-RBCs) were determined. Red cells from four patients with sickle hemoglobinopathies were treated with 50 mM sodium cyanate for 2 hr (37 degrees C), glycerolized and frozen (-80 degrees C) for 62-153 days. The mean in vitro loss of S-RBCs from the combination of cyanate treatment and cryopreservation was 23.6% ( +/- 3.5 SD). The 2,3-diphosphoglycerate content of the thawed cells did not change significantly. However, ATP levels decreased to about 50% of the corresponding values in fresh, untreated S-RBCs. Despite this decrease in ATP, the mean intravascular survival of the frozen cyanated cells nearly doubled. At the high concentration of cyanate used, the oxygen affinity of S-RBCs increased markedly: Their mean P50 was 13.1 mm Hg ( +/- 1.9SD). The gelation of HbS at zero pO2 was also markedly inhibited in the one sample of cyanate-treated S-RBCs examined. Clinical studies to determine the efficacy of autologous transfusions with extensively carbamylated, cryopreserved S-RBCs should be considered.

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