1. A case of chronic hemolytic anemia, present since infancy, in a young Negro male has been reported. There were no hematologic abnormalities in the six family members who were examined.
2. The unusual features in this patient were normal red blood cell fragility, the absence of spherocytosis and marked punctate basophilia. These findings appear to conform to the syndrome of hereditary hemolytic anemia without spherocytosis described by Haden. This is the first reported case of this syndrome in a Negro.
3. Examination of material obtained by splenic puncture revealed that:
a. Stippled cells were much fewer in the spleen aspirate than in the peripheral blood.
b. Basophilic granules were found engulfed by the reticulo-endothelial cells of the spleen.
These data suggest as a possible explanation for the hemolytic mechanism that the spleen selectively destroys the abnormal stippled erythrocytes. The mechanism of formation of these erythrocytes remains obscure.