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(Pediatrics in Review. 1985;6:201-208.)
© 1985 American Academy of Pediatrics
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Congenital Hemolytic Anemia

Joseph E. Addiego Jr MD1
Deborah Hurst MD1
Bertram H. Lubin MD1
 1 Department of Hematology/Oncology, Bruce Lyon Memorial Research Laboratory, Children's Hospital Medical Center, 51st and Grove Sts, Oakland, CA 94609

Anemia due to premature destruction of RBCs is classified as hemolytic, and may be secondary to acquired or inherited (congenital) abnormalities in RBC membrane, hemoglobin, or metabolism. This article reviews the topic of congenital hemolytic anemias and points out a practical approach to diagnosis and therapy.

PATHOPHYSIOLOGY

The primary function of RBCs is to transport oxygen from the lungs to the tissues. The normal RBC circulates within the vascular system for about 120 days. Survival to its full life span depends upon effective metabolism of glucose to generate high-energy phosphates such as adenosine triphosphate (ATP) and to maintain adequate levels of reduced glutathione. ATP is essential for cellular deformability and for function of many RBC enzymes, whereas reduced glutathione protects the cell against damage from oxidants. The water and cation content of the cell is maintained by barriers to permeation, and by transport mechanisms located within the cell membrane. The integrity of the membrane is determined by a group of membrane proteins (skeletal proteins) and by the lipid bilayer surrounding the cell. Genetic or acquired defects of any of these components of the RBC may lead to cellular damage and result in hemolytic anemia.

CLINICAL FEATURES

Hemolytic anemia should be suspected in any child with anemia, jaundice, and splenomegaly.






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Pediatrics Pediatrics in Review
Copyright © 1985 by the American Academy of Pediatrics.