Red cell life span after splenectomy in hereditary spherocytosis.

Despite the persistence of spherocytosis after splenectomy in hereditary spherocytosis, it has usually been assumed that red cell life span returns completely to normal after this treatment. Diisopropyl fluorophosphate. DF(32)P, a noneluting red cell label, was given intravenously to 11 patients in five unrelated families 2-27 yr after splenectomy for typical hereditary spherocytosis. Hemoglobin ranged from 14.0 to 19.8 g/100 ml in this group and reticulocytes from 1.1 to 2.9%, showing the excellent clinical response to splenectomy. Loss of red cell radioactivity corrected for radiophosphorus decay was linear with time during the 60-70 days of the study. Red cell survival as indicated by this rate of loss was 96 +/- 13 days (range 76-118 days), significantly less than the 123 +/- 14 days observed with the same method in 12 persons with normal red cells (P < 0.0005). I conclude that splenectomy does not eliminate the decreased red cell survival in hereditary spherocytosis. The residual 22% decrease in red cell survival is clinically unimportant, but it must be considered in evaluation of biochemical differences observed in hereditary spherocytic red cells.