Abstract
Mutation in the Beta Globin Gene (HBB) leads to the formation of Hemoglobin S (HbS) and Hemoglobin C (HbC). Co-inheritance of HbS and HbC causes hemoglobin SC disease, a form of hemolytic anemia with a myriad of clinical manifestations. Valine replaces glutamic acid in the 6th position (Glu 6Val) to form HbS and Lysine replaces glutamic acid (Glu6Lys) in HbC. The interaction of HbC with HbS increases the propensity of red blood cells to sickle leading to microvascular occlusion and down-stream end organ complications.
Highlights
Hemoglobin SC disease (HbSC) is the second most common hemoglobin disorder after hemoglobin SS disease (HbSS), with a world-wide prevalence of 55,000 new babies born with the disease every year [3]
These findings indicate that increased blood viscosity and abnormal blood rheology in HbSC individuals contribute to disease pathology
Retinitis Proliferans (RP) or proliferative sickle cell retinopathy is a common complication of HbSC disease, occurring in 30-70% of the patients with the disease compared to 3% of HbSS patients [36,37,38]
Summary
Hemoglobin SC disease (HbSC) is the second most common hemoglobin disorder after hemoglobin SS disease (HbSS), with a world-wide prevalence of 55,000 new babies born with the disease every year [3]. Impaired red cell deformability and blood hyperviscosity has been noted in patients with HbSC disease with retinopathy and otological complications, respectively [23]. Chronic Hemolytic anemia is a hall mark of HbSC disease and individuals with HbSC disease have higher hemoglobin and lower absolute reticulocyte count compared to Sickle Cell Disease patients [26].
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