Hemolytic Mechanism in Sickle Cell-Hgb C Disease.

Abstract

Effect of the spleen on the hemolytic mechanism of sickle cell-Hgb C disease was determined by transfusing erythrocytes from the latter disease into normal individuals with and without a spleen. No significant difference in survival time of the erythrocytes, by either the Ashby technique or by the Cr51 tagged red cell method, could be shown. Autotransfusion of sickle cell-Hgb C red cells, tagged with Cr51, resulted in survival time curves with about the same half-life as in homotransfusion studies. These studies suggest that the spleen is not essential for the hemolysis occurring in sickle cell-Hgb C disease and this disease resembles true sickle cell anemia rather than hereditary spherocytosis as far as the role of the spleen is concerned. Since splenomegaly is frequently found during the crises in sickle cell-Hgb C disease, there may be an additional element of dyssplenism during such crises.