eMedicine Specialties > Hematology > Uncommon RBC Membrane Disorders

Spur Cell Anemia: Treatment & Medication

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

Treatment

Medical Care

Treatment in cases of acanthocytosis is directed at the underlying disease.

  • Anemia can be corrected by red blood cell transfusion. However, the transfused cells become acanthocytic, with shortened life span in the circulation.
  • Supportive care for patients with reversible liver disease is the mainstay of treatment. Abstinence from alcohol use may result in the nearly complete disappearance of acanthocytes in the peripheral blood in patients with mild to moderate alcoholic liver cirrhosis.
  • Patients with abetalipoproteinemia may benefit from dietary measures that include triglyceride restriction and lipid-soluble vitamin supplementation.

Surgical Care

The poor general status of acanthocytic patients limits the use of surgical care.

  • Three cases of spontaneous resolution of spur cell anemia following orthotopic liver transplantation have been reported.
  • Splenectomy may improve the hemolytic anemia. However, these patients are severely ill and, in most cases, cannot undergo surgery.

Consultations

Genetic counseling is offered to families of patients with abetalipoproteinemia and chorea-acanthocytosis syndromes.

Diet

  • Because patients with abetalipoproteinemia cannot absorb triglycerides, a diet restricted in these nutrients may result in significant improvement of symptoms. Supplementation of the diet with lipid-soluble vitamins A, K, E, and D results in further improvement of neurologic and retinal symptoms.
  • Patients should abstain from alcohol use.

Medication

The goals of pharmacotherapy are to reduce morbidity, correct vitamin deficiencies, and prevent complications.

Vitamins

Vitamins are organic substances essential to normal metabolism.


Vitamin E (Vita-Plus E Softgels, Vitec, Aquasol E, Vite E Creme)

Protects polyunsaturated fatty acids in membranes from attack by free radicals and protects red blood cells (RBCs) from hemolysis.

Adult

Recommended dietary allowance (RDA) dose: 8-10 mg/d PO (12-15 IU/d)

Therapeutic dose: 50-2000 IU/d PO

Deficiency: 30-50 mg PO qd (PO dose usually is 4- to 5-times the RDA)

Pediatric

RDA dose: 3-10 mg/d PO

Therapeutic dose: 1-100 mg/kg/d PO

Mineral oil decreases absorption; delays the absorption of iron and increases the effects of anticoagulants

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

May induce vitamin K deficiency; necrotizing enterocolitis may occur with large doses


Phytonadione (AquaMEPHYTON)

Vitamin K is a fat-soluble vitamin absorbed by the gut and stored in the liver. Necessary for the function of clotting factors in the coagulation cascade. Used to replace the essential vitamins not obtained in sufficient quantities in the diet or to further supplement levels.

Adult

10 mg PO/IV/IM/SC should replete liver stores

Pediatric

1 mg IM

The effects of warfarin, sodium, and dicumarol are antagonized.

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Ineffective in hereditary hypoprothrombinemia; rapid infusion may result in flushing and a feeling of constriction in the chest; relatively nontoxic, even in massive doses

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
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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].

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Further Reading

Related eMedicine Topics

Clinical Trial

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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

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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.

 
 
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