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Sickle cell disease (SCD) is caused by an autosomal recessive mutation in the β-globin chain of hemoglobin A (HbA), causing the mutated hemoglobin S (HbS). In a vaso-occlusive crisis (VOC), sickled red blood cells (RBCs) increase the viscosity of blood and cause microvascular occlusion, contributing to hypoxia, acidosis and further sickling. The clinical manifestations of SCD are diverse and are related to chronic hemolysis with periodic episodes of vascular occlusion that can affect nearly every organ system.

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