eMedicine Specialties > Hematology > Uncommon RBC Membrane Disorders
Spur Cell Anemia: Treatment & Medication
Updated: Nov 10, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
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
Documented hypersensitivity
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.
Documented hypersensitivity
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
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].
Bohlega S, Riley W, Powe J, Baynton R, Roberts G. Neuroacanthocytosis and aprebetalipoproteinemia. Neurology. Jun 1998;50(6):1912-4. [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].
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].
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].
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].
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].
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].
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].
Further Reading
Related eMedicine Topics
- Acanthocytosis [in the Pediatrics: General Medicine section]
- Alcoholic Hepatitis [in the Gastroenterology section]
- Anemia
- Cirrhosis [in the Gastroenterology section]
- Hemolytic Anemia
- Vitamin A Deficiency [in the Endocrinology section]
- Vitamin D Deficiency and Related Disorders [in the Endocrinology section]
- Vitamin E Deficiency [in the Endocrinology section]
- Vitamin K Deficiency [in the Endocrinology section]
Clinical Trial
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
Treatment & Medication: Spur Cell Anemia