Hereditary Spherocytosis: Observations on Hemolytic Mechanisms and Iron Metabolism

1. Two healthy patients with hereditary spherocytosis were phlebotomized until they developed iron deficiency and the erythrocytes became hypochromic. The hereditary spherocytes were no longer spheroidal: they became thin, and the fragility tests improved. However, the life span of the cells in the circulation was not improved. Later, splenectomy corrected the hemolytic disease.

2. In both patients, prior to the experiment, the hemolytic disease was compensated. There was no anemia despite the rapid turnover of red cells. In one of the patients, whose average red cell life span was only five days, the output of hemoglobin must have been exceedingly high. It was computed to be 135 Gm. per day, or 20 times the normal rate.

3. Some aspects of iron metabolism in hereditary spherocytosis are discussed.

4. The shape of the red cell in HS does not appear to be responsible for its premature destruction by the spleen. Iron deficiency corrects the spherocytosis, but it does not correct the hemolytic disease. Splenectomy corrects the hemolytic disease, but it does not correct the spherocytosis.