Splenectomy in the Treatment of Hemolytic Anemia

In 113 patients with hemolytic anemia splenectomy was performed, without mortality and with minimal morbidity. Fifty-three patients with congenital spherocytosis and two with congenital elliptocytosis had postoperative increases in hematocrit to normal or near-normal levels. Three patients with pyruvate kinase deficiency and three with thalassemia variants were improved. Splenectomy in 52 patients with autoimmune hemolytic anemia resulted in an excellent response in 64% (no further steroid therapy) and an improved status in another 21% (prednisone requirements, 15 mg/day or less). For conditions other than congenital spherocytosis, in which splenectomy is uniformly of value, a decision to remove the spleen should be based on severity of the hemolytic process, failure to respond to other therapies, and the potential for achieving significant improvement in anemia and other associated cytopenias.