Abstract

1. A case report and special studies of a 14-year-old girl with a congenital hemolytic anemia are reported.

2. Fourteen per cent of her erythrocytes contained unusual inclusion bodies.

3. In addition, the child has been known to pass dark urine since the age of 2½ years. The pigment probably belongs to the bilifuscin and mesobilifuscin group.

4. It is believed that the syndrome is probably caused by an inborn error in erythrocytic metabolism.

5. It has been proposed that the syndrome be named "congenital hemolytic anemia with abnormal pigment metabolism and red cell inclusion bodies."

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