Role of the Spleen and Effect of Splenectomy in Sickle Cell Disease

The survival of sickle cell anemia and sickle-cell/Hb-C erythrocytes was determined by the radioactive chromium method, both in their parent circulations and in the circulations of compatible normal recipients.

In sickle cell anemia patients with splenomegaly the average erythrocyte survival time (T½) was found to be 3.7 days. After splenectomy T½ increased to an average of 11.4 days.

In sickle cell anemia patients without splenomegaly the average T½ was found to be 10 days. In five instances an average T½ of 9.2 days was found, whereas the average T½ for the same cells in the circulations of normal recipients was 4.4 days.

In sickle-cell/Hb-C disease the average T½ was 15.7 days. Following splenectomy in two patients, T½ was unchanged in one and increased in the second. T½ was shortened in two of three instances when these cells were transfused into normal recipients, but the data are insufficient to permit conclusions to be drawn.

The role of the spleen in hemolysis is discussed briefly and it is concluded that the hemolytic process in sickle cell anemia is accelerated in the presence of a spleen. This finding is compatible with the diminution in the severity of sickle cell anemia frequently recognized in the adult patient whose spleen has atrophied, and in the child following splenectomy. The validity of the chromate-tagging method for determining erythrocyte survival is discussed. Splenectomy was performed in children in whom the erythrocyte survival was shortened (T½ less than 6 days).