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Defining Sickle Cell Disease
Sickle cell disease (SCD) is the consequence of homozygosity for a single amino acid change in the beta-globin chain that results in structurally abnormal hemoglobin S, or by compound heterozygosity for hemoglobin S and another β-globin chain abnormality, typically hemoglobin C or beta-0 thalassemia. Hemoglobin SS (HbSS) and HbS beta-0 thalassemia present in a similar clinical manner and are commonly referred to together as sickle cell anemia (SCA), which is the most severe subtype of SCD.
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The sickling process causes secondary changes in cell shape, size, cation and water content, and membrane structure that contribute to the impairment of intrinsic cell deformability. ....

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