eMedicine Specialties > Hematology > Uncommon RBC Membrane Disorders

Spur Cell Anemia: Differential Diagnoses & Workup

Author: Issam Makhoul, MD, Associate Professor, Department of Medicine, Division of Hematology/Oncology, University of Arkansas for Medical Sciences
Coauthor(s): Mansoor Javeed, MD, FACP, Clinical Assistant Professor of Medicine, University of California Davis; Consultant, Sierra Hematology-Oncology Medical Center; James O Ballard, MD, Kienle Chair for Humane Medicine, Professor, Departments of Humanities, Medicine, and Pathology, Division of Hematology/Oncology, Milton S Hershey Medical Center, Pennsylvania State University College of Medicine
Contributor Information and Disclosures

Updated: Nov 10, 2009

Differential Diagnoses

Other Problems to Be Considered

Echinocytes and keratocytes should be considered in the differential diagnosis of spur cell anemia. Echinocytes or burr cells are spiculated red blood cells with uniform, narrow, spikelike surface projections. Spur cells have fewer spicules, and the spicules vary more in size than echinocytes. Keratocytes are acanthocytelike cells with bizarre shapes and horny projections.

Other causes of hemolytic anemia in severe liver disease include the following:

  • Hemolytic anemia with echinocytes
  • Severe hypophosphatemia
  • Splenomegaly-related hemolytic anemia with spherocytosis
  • Zieve syndrome

Workup

Laboratory Studies

The laboratory workup in cases of spur cell anemia include the following:

  • Complete blood cell (CBC) 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.
  • 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 Image 1), particularly if obstructive jaundice is present.
      Acanthocytes with target cells in a patient with ...

      Acanthocytes with target cells in a patient with advanced liver disease.

      [ CLOSE WINDOW ]
      Acanthocytes with target cells in a patient with ...

      Acanthocytes with target cells in a patient with advanced liver disease.

  • Liver function tests
    • Hyperbilirubinemia, predominantly indirect bilirubin, is present and its increase parallels the hemolysis.
    • Synthetic liver function is decreased, as attested 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.

Procedures

  • Intestinal biopsy in abetalipoproteinemia: This procedure reveals the presence of fat droplets within the mucosal cells.

More on Spur Cell Anemia

Overview: Spur Cell Anemia
Differential Diagnoses & Workup: Spur Cell Anemia
Treatment & Medication: Spur Cell Anemia
Follow-up: Spur Cell Anemia
Multimedia: Spur Cell Anemia
References
Further Reading
[ CLOSE WINDOW ]

References

  1. Doll DC, Doll NJ. Spur cell anemia. South Med J. Oct 1982;75(10):1205-10. [Medline].

  2. 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].

  3. Shohet SB, Ness PM. Hemolytic anemias. Failure of the red cell membrane. Med Clin North Am. Sep 1976;60(5):913-32. [Medline].

  4. Cooper RA. Hemolytic syndromes and red cell membrane abnormalities in liver disease. Semin Hematol. Apr 1980;17(2):103-12. [Medline].

  5. 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].

  6. 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].

  7. 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].

  8. Wong P. A basis of the acanthocytosis in inherited and acquired disorders. Med Hypotheses. 2004;62(6):966-9. [Medline].

  9. 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].

  10. 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].

  11. Bohlega S, Riley W, Powe J, Baynton R, Roberts G. Neuroacanthocytosis and aprebetalipoproteinemia. Neurology. Jun 1998;50(6):1912-4. [Medline].

  12. 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].

  13. Goel A, Kumar J DI, Nair S, et al. Education and imaging. Hepatobiliary and pancreatic: spur cell anemia associated with alcoholic cirrhosis. J Gastroenterol Hepatol. Sep 2008;23(9):1463. [Medline].

  14. Hardie RJ, Pullon HW, Harding AE, et al. Neuroacanthocytosis. A clinical, haematological and pathological study of 19 cases. Brain. Feb 1991;114 (pt 1A):13-49. [Medline].

  15. Katsube T, Shimono T, Ashikaga R, et al. Demonstration of cerebellar atrophy in neuroacanthocytosis of 2 siblings. AJNR Am J Neuroradiol. Oct 22 2008;epub ahead of print. [Medline].

  16. Pascoe A, Kerlin P, Steadman C, et al. Spur cell anaemia and hepatic iron stores in patients with alcoholic liver disease undergoing orthotopic liver transplantation. Gut. Aug 1999;45(2):301-5. [Medline]. [Full Text].

  17. Rodrigues GR, Walker RH, Bader B, et al. Chorea-acanthocytosis: report of two Brazilian cases. Mov Disord. Oct 30 2008;23(14):2090-3. [Medline].

  18. Rubio JP, Danek A, Stone C, et al. Chorea-acanthocytosis: genetic linkage to chromosome 9q21. Am J Hum Genet. Oct 1997;61(4):899-908. [Medline]. [Full Text].

[ CLOSE WINDOW ]

Further Reading

Related eMedicine Topics

Clinical Trial

[ CLOSE WINDOW ]

Keywords

spur cell anemia, spur cell hemolysis, acanthocyte cell hemolytic anemia, acanthocytosis, spur cell hemolytic anemia, neuroacanthocytosis, abetalipoproteinemia, chorea acanthocytosis syndrome, choreoacanthocytosis, chorea-acanthocytosis syndrome, McLeod phenotype

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Issam Makhoul, MD, Associate Professor, Department of Medicine, Division of Hematology/Oncology, University of Arkansas for Medical Sciences
Issam Makhoul, MD is a member of the following medical societies: American Society of Clinical Oncology and American Society of Hematology
Disclosure: Nothing to disclose.

Coauthor(s)

Mansoor Javeed, MD, FACP, Clinical Assistant Professor of Medicine, University of California Davis; Consultant, Sierra Hematology-Oncology Medical Center
Mansoor Javeed, MD, FACP is a member of the following medical societies: American College of Physicians and Pennsylvania Medical Society
Disclosure: Nothing to disclose.

James O Ballard, MD, Kienle Chair for Humane Medicine, Professor, Departments of Humanities, Medicine, and Pathology, Division of Hematology/Oncology, Milton S Hershey Medical Center, Pennsylvania State University College of Medicine
James O Ballard, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American Heart Association, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Medical Editor

Koyamangalath Krishnan, MD, FRCP, FACP, Paul Dishner Endowed Chair of Excellence in Medicine, Professor of Medicine and Chief of Hematology-Oncology, Program Director, Hematology-Oncology Fellowship, James H Quillen College of Medicine at East Tennessee State University
Koyamangalath Krishnan, MD, FRCP, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, American Society of Hematology, and Royal College of Physicians
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Marcel E Conrad, MD, (Retired) Distinguished Professor of Medicine, University of South Alabama
Marcel E Conrad, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association of Blood Banks, American Chemical Society, American College of Physicians, American Physiological Society, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Association of Military Surgeons of the US, International Society of Hematology, Society for Experimental Biology and Medicine, and Southwest Oncology Group
Disclosure: No financial interests None None

CME Editor

Rajalaxmi McKenna, MD, FACP, Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.