Congenital Hemolytic Disease Associated with Red Cell Inclusion Bodies, Abnormal Pigment Metabolism and an Electrophoretic Hemoglobin Abnormality

1. A fourth case of the syndrome of congenital hemolytic anemia with abnormal pigment metabolism and red cell inclusion bodies following splenectomy is described.

2. In this case an abnormality was found on paper and starch electrophoresis of the red cell hemolysate at pH 8.6. A similar abnormality has not been reported previously.

3. An increased rate of erythrocyte autohemolysis was found during in vitro incubation, Partial correction of this defect occurred in the presence of glucose or purine ribosides.

4. Genetic transmission of the defect could not be demonstrated.