Pathophysiology of β-thalassemia. Absent or reduced β-globin production leads to an unbalanced excess of α-globin chains, which then trigger cascade of events through the generation of ROS, resulting in hemolysis of mature red blood cells and destruction of immature erythroid precursors in the bone marrow (ineffective erythropoiesis). AHSP, α hemoglobin stabilizing protein; HSP70, heat shock protein 70.