Observations on the anemia of cryptogenetic splenomegaly. I. Hemolysis

Excessive hemolysis has been shown to occur in patients with cryptogeneticsplenomegaly. This is corrected by splenectomy.

The survival of normal red cells transfused to such patients is greatlyreduced. Following splenectomy normal red cells so transfused survive normally.

The survival of red cells from such patients upon transfusion to healthyrecipients, 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 butotherwise healthy recipients the survival time is normal.

Study of osmotic fragility before splenectomy demonstrates the existenceof a mixed population showing both increased and decreased fragility tohypotonic saline. Following splenectomy there emerges a population withincreased resistance to hypotonic saline.

It is concluded that prior to splenectomy the pathologic spleen exerts aneffect upon circulating erythrocytes including transfused normal red cellswhich results in marked reduction in their survival. Following splenectomya second defect in the erythrocytes becomes apparent and this also resultsin reduction in life-span, although the presence of the spleen would appearnecessary for this reduction to occur. It is suggested that this second defectdevelops as the result of liver damage.

Submitted on February 12, 1957 Accepted on December 25, 1957