Definition: an abnormal Hb with substitution of a valyl residue for a glutamyl residue at the sixth position of the ß chain; the formula is a2Aß2S, or, more specifically, a2Aß26Glu?Val. Heterozygous state: sickle cell trait, no anemia, Hb S 20–45% of total, the rest Hb A. Homozygous state: sickle cell anemia, Hb S 75–100% of total, the rest Hb F or Hb A2.
Synonym(s): sickle cell hemoglobin
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© Copyright 2018 Wolters Kluwer. All Rights Reserved. Review date: Sep 19, 2016.