Observations on the Anemia of Cryptogenetic Splenomegaly. I. Hemolysis

Abstract

Excessive hemolysis has been shown to occur in patients with cryptogenetic splenomegaly. This is corrected by splenectomy. The survival of normal red cells transfused to such patients is greatly reduced. Following splenectomy normal red cells so transfused survive normally. The survival of red cells from such patients upon transfusion to healthy recipients, including a splenectomized recipient, is greatly reduced. Following splenectomy the survival of red cells from such patients upon transfusion to healthy recipients is significantly increased, although still significantly less than normal. However, upon transfusion into splenectomized but otherwise healthy recipients the survival time is normal. Study of osmotic fragility before splenectomy demonstrates the existence of a mixed population showing both increased and decreased fragility to hypotonic saline. Following splenectomy there emerges a population with increased resistance to hypotonic saline. It is concluded that prior to splenectomy the pathologic spleen exerts an effect upon circulating erythrocytes including transfused normal red cells which results in marked reduction in their survival. Following splenectomy a second defect in the erythrocytes becomes apparent and this also results in reduction in life-span, although the presence of the spleen would appear necessary for this reduction to occur. It is suggested that this second defect develops as the result of liver damage.