Hematological disorders including beta-thalassemia major, sickle cell disease and myelodysplastic syndromes are managed with regular blood transfusions. A significant adverse effect of blood transfusions is iron overload. Each unit of blood contains 250 mg of iron, however, humans have no physiological mechanism to excrete excess iron. Over time, the infused iron accumulates causing toxicity. At normal levels of iron, the metal is bound by transferrin in the blood or stored in a non-toxic form as ferritin in the body. However, when the body's storage capacity is exceeded, the excess iron not bound by transferrin is taken up into the liver, heart, and endocrine cells causing severe tissue damage with an increased risk of cardiac toxicity and death.
Thalassemia patient who
receives chronic blood transfusions