Abstract
Quantitative determinations of iron kinetics and hemoglobin synthesis were made on five patients with sickle cell-hemoglobin C disease. The anemia was mild in all patients but one who had the hemoglobin of 10.1 Gm. per cent. All patients were in a steady state during the period of this study.
The ferrokinetic determinations demonstrated a hemolytic process in all cases. The mean erythrocyte life span in these patients was 18, 20, 29, 46 and 56 days respectively (normal range, 110-130 days). The hemoglobin synthesis was increased in all. Reduced to terms of daily hemoglobin production per liter of blood, the values were 2.2 Gm., 2.9 Gm., 3.6 Gm., 5.8 Gm. and 6.6 Gm. The latter figure represents a five fold increase over the normal mean value of daily hemoglobin synthesis of 1.3 Gm. (normal range, 1.0 to 1.6 Gm. of hemoglobin per liter of blood per day).
The results of the in vivo organ counts demonstrated a significant degree of sequestration of red blood cells in the spleen of two patients. The question of advisability of splenectomy in such patients was discussed.
