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Hemoglobin C
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. | |
