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hemoglobin C

 

Definition: an abnormal Hb with substitution of lysyl residue for glutamyl at the 6th position of the ß chain, of formula a22Aß26Glu?Lys, this type reduces the normal plasticity of erythrocytes. Heterozygotes: Hb C trait, about 28–44% of total Hb is Hb C, no anemia. Homozygotes: nearly all Hb is Hb C, moderate normocytic hemolytic anemia. Patients heterozygous for both Hb C and Hb S (Hb SC disease) and for Hb C and thalassemia are known, and have atypical hemolytic anemias; sickling is enhanced in Hb SC disease.

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© Copyright 2018 Wolters Kluwer. All Rights Reserved. Review date: Sep 19, 2016.

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