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hemoglobin S

 

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 a22S, or, more specifically, a226Glu?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 2017 Wolters Kluwer. All Rights Reserved. Review Date: Sep 19, 2016.
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