Erythrocyte lipid alterations in pediatric cholestatic liver disease: spur cell anemia of infancy.
Spur cell anemia of liver disease is a hemolytic process characterized by spiculated erythrocytes and an elevated red cell membrane cholesterol/phospholipid (C/PL) molar ratio. This form of anemia is associated almost exclusively with adults in the advanced stages of alcoholic cirrhosis. We were therefore surprised to identify two unrelated infants with cholestatic liver disease and hemolytic anemia who had spiculated erythrocytes as the major abnormal cell form on peripheral smear. Erythrocyte membrane cholesterol and phospholipid determinations from these patients were compared with six infants with extrahepatic biliary atresia and target-shaped erythrocytes and with five normal adults. Erythrocyte C/PL molar ratio distinguished target cells from normal erythrocytes (p less than 0.01). The spur cell patients' erythrocyte C/PL molar ratios were clearly greater than either target cell patients or normal controls (1.30 vs. 1.02 vs. 0.84). Both patients' spur cell anemia resolved and target cells became the major abnormal erythrocyte form. These studies identify a transient form of spur cell anemia associated with infantile cholestatic liver disease. The factors leading to the formation of spur cell anemia in infancy require further investigation.
D000050:Acanthocytes / Q000378:metabolism*; D000743:Anemia, Hemolytic / Q000097:blood / Q000150:complications* / Q000378:metabolism; D001706:Biopsy; D002675:Child, Preschool; D002780:Cholestasis, Intrahepatic / Q000097:blood / Q000150:complications* / Q000473:pathology; D002784:Cholesterol / Q000097:blood; D004910:Erythrocyte Membrane / Q000378:metabolism; D004913:Erythrocytes, Abnormal / Q000378:metabolism*; D005260:Female; D006801:Humans; D007223:Infant; D008099:Liver / Q000473:pathology; D008297:Male; D008563:Membrane Lipids / Q000378:metabolism*; D010743:Phospholipids / Q000097:blood
D008563:Membrane Lipids; D010743:Phospholipids; D002784:Cholesterol