Improvement of sickle cell anemia by iron‐limited erythropoiesis

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We report the hematologic and clinical features of four adult patients (Pts.) with sickle cell anemia and iron‐limited erythropoiesis. Two of the Pts. had spontaneous iron deficiency (chronic Gl bleeding, low‐grade hemoglobinuria). In the other two Pts. iron restriction was induced by periodic RBC aphereses as part of a pilot protocol designed to decrease intracellular HbS polymerization by MCHC reduction. Iron‐limited erythropoiesis was defined by reduction in red cell indices (MCV range 60.4–67 fl) in the presence of low serum ferritin (range <10–20 ng/ml). In these Pts. iron restriction did not cause clinically significant worsening of the anemia (Hb 7.8–9.0 g/dl). In two Pts. the anemia actually improved. Other hematologic effects of iron restriction were: decreased MCHC, reticulocyte count, RDW, and dense cells. A reduced hemolytic rate was suggested by a lowering of serum bilirubin and LDH. In one of the Pts. the 51Cr RBC T1/2 survival increased from 12 to 16 days. The intracellular HbS polymer fractions (fp) were determined at 25% O2 by Csat and with the use of the conservation of mass equation. The baseline fp values ranged from 0.48–0.53. After iron restriction they ranged from 0.33–0.48. The fp decreased even though iron‐limited erythropoiesis also lowered the Hb F concentration in three of our Pts. In one of the two Pts. with induced iron depletion, hospitalization days for pain crises decreased from an average of 4.5 days/month (2 year baseline period) to an average of 0.5 days/month in the 3 year follow‐up after iron depletion. The second patient with induced iron restriction experienced the rapid healing of a leg ulcer. Controlled iron restriction should be explored as a therapeutic strategy in selected SS patients. © 1994 Wiley‐Liss, Inc. Copyright © 1994 Wiley‐Liss, Inc., A Wiley Company

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