2004 Volume 18 Issue 3 Pages 140-145
Hereditary spherocytosis (HS) is a disease based on abnormalities of the red cell membrane that shows clinical heterogeneity. To assess the relationship between the severity of red cell membrane abnormalities and clinical features, 28 HS patients underwent red cell membrane analysis by SDS polyacrylamide gel electrophoresis (PAGE), as well as hematology tests and clinical examination. A good correlation was found between the hemoglobin level and the amount of membrane protein band 3 or the size of the spleen. Patients with low levels of band 3 tended to have a low hemoglobin and a large spleen was also associated with a low hemoglobin. These data suggest that the severity of anemia is related to the amount of membrane band 3 protein in HS patients. Splenectomized patients had low levels of band 3 before splenectomy, except for 3 patients with near normal band 3 levels. From these data, patients with a low amount of band 3 should receive splenectomy because these patients also have severe anemia.
