Sickle cell disease (SCD) is one of the most important monogeneic diseases in the world, causing significant morbidity and mortality and consuming substantial healthcare resources.1 SCD results from a single amino acid substitution in the β-globin chain, leading to sickle hemoglobin (HbS). In oxygen-limited settings, HbS forms polymers that lead to the characteristic sickle-shaped erythrocytes and also to changes in the erythrocyte membrane. The pathological features of SCD result from a combination of vaso-occlusive events precipitated both by abnormal erythrocyte morphology and the increased expression of adhesion molecules on its outer membrane; accelerated hemolysis that can deplete nitric oxide and that leads to anemia; and a high level of endogenous inflammatory activation associated …
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