C5 and Tangier disease: In addition to residual transfusion dependency, patients treated with C5 inhibitors develop EVH, leading to iron overload and retention due to increased iron absorption from anemia-driven erythropoiesis due to hemolysis, decreased renal iron excretion due to reduced IVH-mediated hemosiderinuria, and EVH-induced iron accumulation in the reticuloendothelial system [20].