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Spur cell anemia associated with primary biliary cirrhosis

Spur cell anemia associated with primary biliary cirrhosis A 61‐year‐old‐female presented with jaundice, massive ascites, and hepatic encephalopathy. She had no history of alcohol consumption, but had been diagnosed with primary biliary cirrhosis 8 years earlier. Laboratory tests indicated the following: Hb level 6.2 g/dL (reference value [RV], 12‐16.5 g/dL), WBC count 10.7 × 10 9 /L (RV, 4 × 10 9 ‐10 × 10 9 /L), PLT count 51 × 10 9 /L (RV, 150 × 10 9 ‐400 × 10 9 /L), reticulocyte count 318 × 10 9 /L (RV, 40 × 10 9 ‐120 × 10 9 /L), cholesterol level 2.09 g/L (RV, 1.5‐2.6 g/L), decreased HDL 0.22 g/L (vascular protection threshold, >0.6 g/L), triglycerides 1.2 g/L (RV, 0.6‐1.5 g/L), increased LDL 2.79 g/L, AST 60 UI/mL (RV, <31 UI/mL), ALT 25 UI/mL (RV, <34 UI/mL), alkaline phosphatase 138 UI/L (RV, 30‐120 UI/L), total bilirubin 199 mg/mL (RV, 1‐14 mg/mL), coagulation factor V 0.45 (RV, >0.7), LDH 968 UI/L (RV, <248 UI/L), and haptoglobin <0.08 g/L (RV, 0.3‐2 g/L). The DAT was negative. The blood smear (see figure, blood cell film [May‐Grünwald‐Giemsa ×1000]) demonstrated abundant spur cells in a background of moderate anisopoikilocytosis without schistocytes. Spur cells are acanthocytes with cytoplasmic projections irregularly distributed over the surface of the RBCs. RBC survival is shortened due to splenic sequestration and destruction. Changes in serum lipids affect the composition of the lipids of the RBC membrane with imbalance in cholesterol/phospholipid ratio. The membrane fluidity decreases as the cholesterol content increases leading to an irreversible rigid transformation and peculiar shapes as in our case. Spur cell formation is enhanced in chronic liver disease due to: 1) impairment of the liver's ability to esterify cholesterol, causing free cholesterol to bind to the RBC membrane and increasing its surface area; 2) the stagnation of sterols on the RBC membrane; 3) changes in plasma lecithin‐cholesterol acyltransferase concentrations; and 4) the retention of bile acids. It has also been suggested that spur cells were acanthocytes remodeled by the spleen, whereby the spicules become more blunt and the associated membrane loss makes the cell more spherocytic (spheroacanthocytes). Spur cell anemia is an uncommon acquired nonimmune hemolytic anemia associated with severe liver disease. Identifying such cells can be important to rule out DAT‐negative immune hemolytic anemia. Most patients have advanced liver failure related to alcoholic cirrhosis. Primary biliary cirrhosis is a rare cause. There is no effective therapy except liver transplantation, but the prognosis remains poor. CONFLICT OF INTEREST No conflict of interest for both authors. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Transfusion Wiley

Spur cell anemia associated with primary biliary cirrhosis

Martin, Michèle; Lesesve, Jean François
Transfusion , Volume 53 (2) – Jan 1, 2013
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References (3)

Publisher
Wiley
Copyright
Copyright © 2013 AABB
ISSN
0041-1132
eISSN
1537-2995
DOI
10.1111/j.1537-2995.2012.03733.x
pmid
23384101
Publisher site
See Article on Publisher Site

Abstract

A 61‐year‐old‐female presented with jaundice, massive ascites, and hepatic encephalopathy. She had no history of alcohol consumption, but had been diagnosed with primary biliary cirrhosis 8 years earlier. Laboratory tests indicated the following: Hb level 6.2 g/dL (reference value [RV], 12‐16.5 g/dL), WBC count 10.7 × 10 9 /L (RV, 4 × 10 9 ‐10 × 10 9 /L), PLT count 51 × 10 9 /L (RV, 150 × 10 9 ‐400 × 10 9 /L), reticulocyte count 318 × 10 9 /L (RV, 40 × 10 9 ‐120 × 10 9 /L), cholesterol level 2.09 g/L (RV, 1.5‐2.6 g/L), decreased HDL 0.22 g/L (vascular protection threshold, >0.6 g/L), triglycerides 1.2 g/L (RV, 0.6‐1.5 g/L), increased LDL 2.79 g/L, AST 60 UI/mL (RV, <31 UI/mL), ALT 25 UI/mL (RV, <34 UI/mL), alkaline phosphatase 138 UI/L (RV, 30‐120 UI/L), total bilirubin 199 mg/mL (RV, 1‐14 mg/mL), coagulation factor V 0.45 (RV, >0.7), LDH 968 UI/L (RV, <248 UI/L), and haptoglobin <0.08 g/L (RV, 0.3‐2 g/L). The DAT was negative. The blood smear (see figure, blood cell film [May‐Grünwald‐Giemsa ×1000]) demonstrated abundant spur cells in a background of moderate anisopoikilocytosis without schistocytes. Spur cells are acanthocytes with cytoplasmic projections irregularly distributed over the surface of the RBCs. RBC survival is shortened due to splenic sequestration and destruction. Changes in serum lipids affect the composition of the lipids of the RBC membrane with imbalance in cholesterol/phospholipid ratio. The membrane fluidity decreases as the cholesterol content increases leading to an irreversible rigid transformation and peculiar shapes as in our case. Spur cell formation is enhanced in chronic liver disease due to: 1) impairment of the liver's ability to esterify cholesterol, causing free cholesterol to bind to the RBC membrane and increasing its surface area; 2) the stagnation of sterols on the RBC membrane; 3) changes in plasma lecithin‐cholesterol acyltransferase concentrations; and 4) the retention of bile acids. It has also been suggested that spur cells were acanthocytes remodeled by the spleen, whereby the spicules become more blunt and the associated membrane loss makes the cell more spherocytic (spheroacanthocytes). Spur cell anemia is an uncommon acquired nonimmune hemolytic anemia associated with severe liver disease. Identifying such cells can be important to rule out DAT‐negative immune hemolytic anemia. Most patients have advanced liver failure related to alcoholic cirrhosis. Primary biliary cirrhosis is a rare cause. There is no effective therapy except liver transplantation, but the prognosis remains poor. CONFLICT OF INTEREST No conflict of interest for both authors.

Journal

Transfusion – Wiley

Published: Jan 1, 2013

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