HbSC disease

atypical sickle cell disease, caused by double heterozygosity

Description
In HbSC, one &#946;-globin gene encodes a chain leading to formation of hemoglobin S (HbS) and the other &#946;-globin gene encodes a &#946;-chain leading to formation of hemoglobin C (HbC). Almost equal parts of HbS and HbC are found in the red cells. HbC, unlike the normal adult Hb (HbA), can participate in the polymerization process with HbS, thus leading to atypical hemolytic anemias with enhanced sickling. See also HbSD