Exchange Transfusion in a Neonatal Crisis of Congenital Hemolytic Anemia

Abstract

Hereditary spherocytosis is a congenital hemolytic disease which is characterized by familial transmission, moderate anemia, and decreased red blood cell survival time; red blood cells which demonstrate a spherical configuration and increased mechanical and osmotic fragility; jaundice, splenomegaly, and marked improvement following splenectomy. Despite persistence of the basic defect in the erythrocytes, splenectomy "promptly, completely, and permanently relieves anemia and jaundice in nearly all cases of hereditary spherocytosis." 2 Some workers have reported an increased incidence of severe and often fulminating infections following splenectomy in early childhood, suggesting decreased resistance to infection after splenectomy.2,3 One suggests use of more rigid criteria for splenectomy and close postoperative supervision.