Abstract

Cyanate reacts with the amino-terminal valine residues of hemoglobin S and prevents the sickling in vitro of 50-80% of the erythrocytes from patients with sickle-cell disease. The purpose of the studies reported here was to determine whether this anti-sickling effect would increase the survival of cyanate-treated cells that were returned to the patient. In seven subjects with sickle-cell disease, the mean 50% survival of (51)Cr-labeled sickle erythrocytes was increased from 9.9 to 20.7 days (normal 25-35 days) after treatment of the cells in vitro with sodium cyanate. These results provide evidence that the antisickling effect of cyanate observed in vitro is retained in vivo, and strengthen the rationale for further investigation of cyanate as a possible therapeutic agent in sickle-cell disease.

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