Approach Considerations
Complete blood count
Test findings reveal variable degrees of anemia, with the hematocrit commonly between 15% and 20%. White blood cell and platelet counts may be normal; however, due to the severe and advanced liver disease, they are decreased in most cases.
Reticulocyte count
An increase in the reticulocyte count depends on the degree of the anemia, but it is usually greater than 5%. In certain cases, the reticulocyte count may be decreased as a result of concomitant folate deficiency.
Liver function tests
Hyperbilirubinemia, predominantly indirect bilirubin, is present, and its increase parallels the hemolysis. Synthetic liver function is decreased, as evidenced by low levels of albumin and fibrinogen and prolongation of the prothrombin time (PT) and activated partial thromboplastin time (aPTT).
Plasma lipids
This study is helpful in screening suspected cases of abetalipoproteinemia. Serum cholesterol, phospholipid, and triglyceride levels are very low. Lipoprotein electrophoresis reveals the absence of beta-lipoproteins.
Blood typing
Kell antisera react poorly with red blood cells, white blood cells, or both in the McLeod phenotype.
Serum creatine kinase
In McLeod syndrome, the creatine kinase levels are increased.
Intestinal biopsy in abetalipoproteinemia
This procedure reveals the presence of fat droplets within the mucosal cells.
Peripheral Blood Film
This study is the mainstay for the diagnosis of spur cell anemia. It reveals the presence of red blood cells with thornlike surface projections, which are variable in size.
Characteristically, a high percentage of acanthocytes is present, equal to or greater than 20% of the erythrocytes observed. In cases of liver disease, target cells also may be seen (see the image below), particularly if obstructive jaundice is present.
Acanthocytes with target cells in a patient with advanced liver disease. Doll DC, Doll NJ. Spur cell anemia. South Med J. Oct 1982;75(10):1205-10. [Medline].
Haruta I, Hashimoto E, Kabutake A, et al. Spur cell anemia associated with a cirrhotic non-alcoholic steatohepatitis patient. Hepatol Res. Jun 2007;37(6):482-5. [Medline].
Shohet SB, Ness PM. Hemolytic anemias. Failure of the red cell membrane. Med Clin North Am. Sep 1976;60(5):913-32. [Medline].
Cooper RA. Hemolytic syndromes and red cell membrane abnormalities in liver disease. Semin Hematol. Apr 1980;17(2):103-12. [Medline].
Cynamon HA, Isenberg JN, Gustavson LP, Gourley WK. Erythrocyte lipid alterations in pediatric cholestatic liver disease: spur cell anemia of infancy. J Pediatr Gastroenterol Nutr. Aug 1985;4(4):542-9. [Medline].
Olivieri O, Guarini P, Negri M, et al. Increased proteolytic activity of erythrocyte membrane in spur cell anaemia. Br J Haematol. Dec 1988;70(4):483-9. [Medline].
Arienti G, Carlini E, Scionti L, Puxeddu E, Brunetti P. Liver alcoholic cirrhosis and spur-cell (acanthocytic) anaemia. A study of erythrocyte ghost composition and fluidity. Scand J Gastroenterol. Dec 1995;30(12):1204-9. [Medline].
Wong P. A basis of the acanthocytosis in inherited and acquired disorders. Med Hypotheses. 2004;62(6):966-9. [Medline].
Redman CM, Russo D, Lee S. Kell, Kx and the McLeod syndrome. Baillieres Best Pract Res Clin Haematol. Dec 1999;12(4):621-35. [Medline].
Terada N, Fujii Y, Ueda H, et al. Ultrastructural changes of erythrocyte membrane skeletons in chorea-acanthocytosis and McLeod syndrome revealed by the quick-freezing and deep-etching method. Acta Haematol. Mar 1999;101(1):25-31. [Medline].
Chitale AA, Sterling RK, Post AB, et al. Resolution of spur cell anemia with liver transplantation: a case report and review of the literature. Transplantation. Apr 15 1998;65(7):993-5. [Medline].
Print
