Anemia with spur cells: a red cell defect acquired in serum and modified in the circulation.
The sera and red cells of three patients with severe liver disease and "spur cells" were studied. In each case the per cent of serum cholesterol which was free (unesterified) was elevated, and the serum lecithin: cholesterol acyltransferase activity was depressed. Lipoproteins with beta mobility were increased, but exhibited immune reactivity with antisera to both alpha- and beta-lipoproteins. Serum bile salt concentrations were markedly elevated and consisted primarily of chenodeoxycholic acid, with small amounts of lithocholic acid present as well.SPUR CELLS MANIFESTED A STRIKING INCREASE IN CHOLESTEROL CONTENT AND IN THE CHOLESTEROL: phospholipid ratio, but a normal osmotic fragility. When incubated in heated normal serum, spur cells lost their excess cholesterol and became spherocytic and osmotically fragile. Conversely, sera from patients with spur cells readily transferred up to one-third of their free cholesterol to normal red cells, causing normal cells to become resistant to osmotic lysis. In addition, these sera caused normal red cells to acquire thorny membrane projections. Cholesterol transfer to normal cells also occurred from normal serum which had previously been incubated with spur cells. Changes in cell cholesterol were induced by all of the lipoprotein fractions of spur serum. When transfused into a patient with spur cells, normal red cells became more resistant to osmotic lysis over the course of 24 hr. However, over the subsequent 7 days they underwent a progressive increase in osmotic fragility. These normal cells, as well as the patient's own cells, had a shortened survival. Correlating with the moderate decrease in the filterability of red cells spurred in vitro, red cell destruction occurred predominently in the spleen. Red cells in this disorder appear to serve as repositories for free cholesterol loosely bound to serum lipoproteins. Cholesterol acquisition by the red cell membrane increases its surface area and causes the red cell to be resistant to osmotic lysis. The associated alteration in red cell shape leads to further changes in the cell membrane during circulation in vivo resulting in the loss of membrane surface area and culminating in the cell's premature destruction in the spleen.
D000740:Anemia / Q000097:blood*; D002462:Cell Membrane; D002784:Cholesterol / Q000097:blood; D002859:Chromium Isotopes; D004912:Erythrocytes / Q000166:cytology* / Q000378:metabolism; D006461:Hemolysis; D006801:Humans; D008074:Lipoproteins / Q000097:blood; D009996:Osmotic Fragility; D010713:Phosphatidylcholines / Q000097:blood; D010743:Phospholipids / Q000097:blood; D013154:Spleen
D002859:Chromium Isotopes; D008074:Lipoproteins; D010713:Phosphatidylcholines; D010743:Phospholipids; D002784:Cholesterol
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