Continuous RBC transfusions in a patient with sickle cell disease

A patient with severe sickle cell anemia received approximately 600 RBC transfusions during a period of 30 years. Continuous prophylactic transfusions were administered for 13 years. During the 13-year period, except for tissue damage already inflicted by sickle cell disease, she was free of symptoms. When a hemoglobin concentration of 12 g/dL was maintained, she was shown to have ineffective erythropoiesis postulated to be due to the inability of her reticulocytes to cross the sinusoidal barrier from marrow to blood. Up to the time of her death at the age of 47 years, there was no evidence that her massive iron overload produced clinical symptoms other than unexplained, although her massive iron overload remains suspect.