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Blood, 1958, Vol. 13, No. 10, pp. 950-958.
© 1958 American Society of Hematology, Inc.


Congenital Hemolytic Anemia with Abnormal Pigment Metabolism and Red Cell Inclusion Bodies: a New Clinical Syndrome

ROBERT D. LANGE 1 and JOSEPH H. AKEROYD 1

1 Department of Hematology, Division of Medicine, Walter Reed Army Institute of Research, Walter Reed Army Medical Center, Washington, D. C.

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 2frac12 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."

Submitted on January 20, 1958
Accepted on May 7, 1958


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