Increased cholesterol and decreased fluidity of red cell membranes (spur cell anemia) in progressive intrahepatic cholestasis.
Progressive hemolytic anemia occurred in a 4 1/2-year-old girl with familial intrahepatic cholestasis; a peripheral smear contained bizarre spiculated "spur" red cells. Analysis of this patient's fresh red cells revealed a 59% increase in cholesterol content with a normal phospholipid content and therefore an increase in the cholesterol/phospholipid molar ratio to 1.35 (normal = 0.92). A similar abnormality of lipid composition was present in serum lipoproteins. The lipid abnormality in red cell membrane was associated with a decrease in membrane fluidity, as assessed by the fluorescence polarization of the hydrophobic probe 1,6-diphenyl-1,3,5-hexatriene. Following incubation with patient's plasma, normal cells acquired a spur-shaped morphology with an associated decrease in osmotic fragility and a 25% increase in cholesterol content. The patient's cells, during incubation with normal plasma, acquired morphologic features of spiculated spherocytes with an increase in osmotic fragility and a 21% decrease in cholesterol content. Chenodeoxycholate and lithocholate were present in markedly elevated concentrations in serum. These studies show that a process identical to spur cell anemia in alcoholic cirrhosis may accompany severe liver disease in children with intrahepatic cholestasis.
D000208:Acute Disease; D000743:Anemia, Hemolytic / Q000209:etiology*; D001647:Bile Acids and Salts / Q000097:blood; D002463:Cell Membrane Permeability; D002635:Chenodeoxycholic Acid / Q000097:blood; D002675:Child, Preschool; D002780:Cholestasis, Intrahepatic / Q000150:complications / Q000235:genetics*; D002784:Cholesterol / Q000097:blood*; D004910:Erythrocyte Membrane; D004913:Erythrocytes, Abnormal / Q000032:analysis*; D005260:Female; D005454:Fluorescence Polarization; D006801:Humans; D066298:In Vitro Techniques; D008074:Lipoproteins / Q000097:blood; D008095:Lithocholic Acid / Q000097:blood; D010743:Phospholipids / Q000097:blood
D001647:Bile Acids and Salts; D008074:Lipoproteins; D010743:Phospholipids; D002635:Chenodeoxycholic Acid; D008095:Lithocholic Acid; D002784:Cholesterol