Iron chelation therapy in sickle cell anemia.

Abstract

AbstractIron stores, ascorbic acid levels, and urinary excretion of iron in response to deferoxamine (DFO) were studied in nine patients with sickle cell anemia who had received 8–55 liters of red blood cells. Ferritin levels ranged from 2, 168 to 6,300 ng/ml and were elevated even in patients who had normal serum iron concentrations. Leukocyte ascorbic acid levels were low in three patients. Urinary iron excretion in response to 2.0 gm DFO administered as a 12‐hour subcutaneous infusion after ascorbic acid supplementation was 13–39 mg/24 hours. With this method of DFO therapy daily, urinary iron excretion would exceed transfusional iron accumulation in eight of nine patients. Following the intramuscular injection of 0.75 gm DFO, urinary iron excretion was 5–16 mg/24 hours. Iron excretion would exceed iron accumulation in only one of nine patients with this method of DFO therapy. Urinary iron excretion in response to 1.5 gm DFO infused intravenously in 18 hours after ascorbic acid supplementation was 11–38 mg/24 hours. In six regularly transfused but nonchelated patients iron excretion in response to intravenous DFO had increased 46–107%, in comparison with previous studies 15–22 months before. These data indicate that negative iron balance can be obtained in regularly transfused patients with sickle cell anemia by the use of overnight subcutaneous infusions of DFO. Urinary iron excretion in response to DFO should be evaluated periodically in appropriate patients with sickle cell anemia to determine the proper time for the institution of chelation therapy.