Abstract
Hypertransfusion programs are being increasingly used in sickle cell anemia (SS dis.). A potential risk of such therapy is transfusional hemosiderosis. It has been assumed that patients with SS dis. acquire large iron burdens from increased GI absorption and sporadic transfusions. There are few data concerning the acquisition of an iron burden in SS dis. Total body iron burden was estimated by 2 indirect methods: 24 hour urinary iron excretion following a 10 mg/Kg IM dose of desferrioxamine (DFO) and the serum ferritin (SF). Serum iron and TIBC were also measured. 20 patients with SS dis. from 3 to 34 years of age were studied. These data were compared with that obtained from patients with thal, major on hypertransfusion programs and thal. intermedia not on regular transfusions. Serum iron and TIBC were normal for all SS dis. patients. DFO-induced urinary iron excretion correlated directly with age, but was normal or only minimally elevated in patients under 20 years of age. Those over 20 had modest increases, but only 2 exceeded 5 mg per day. SF did not correlate with age and were normal in almost half the patients. Elevations in SF were moderate and only rarely exceeded 1000 ng/ml. Patients with thal, major acquire massive iron burdens at an early age as indicated by SF in excess of 2000 ng/ml and very high DFO-induced urinary iron excretions. These data indicate that patients with SS dis. managed by conventional means do not acquire a large total body iron burden through early adult life.
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Obrien, R., Pearson, H. IRON BURDEN IN SICKLE CELL ANEMIA. Pediatr Res 11, 478 (1977). https://doi.org/10.1203/00006450-197704000-00649
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DOI: https://doi.org/10.1203/00006450-197704000-00649