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Acanthocytosis Medication

  • Author: Pedro A de Alarcon, MD; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
Updated: Oct 15, 2015

Medication Summary

Treatment of abetalipoproteinemia includes dietary restriction of triglycerides, supplementation with medium-chain triglycerides, and supplementation of lipid-soluble vitamins A, D, E, and K in high doses. Occasionally, patients have associated iron or folic acid deficiencies, necessitating supplementation with oral iron and folic acid.


Vitamins and Cofactors

Class Summary

These agents are organic substances required by the body in small amounts for various metabolic processes. They are classified as fat-soluble or water-soluble. Vitamins A, D, E, and K are fat-soluble; biotin, folic acid, niacin, pantothenic acid, the B vitamins (ie, B-1, B-2, B-6, B-12), and vitamin C are generally water-soluble. These agents are clinically used for the prevention and treatment of specific vitamin-deficiency states.

Vitamin A (Aquasol A, Palmitate-A 5000)


Required for bone development, growth, night vision, and gonadal function and is a biochemical cofactor.

Ergocalciferol (Drisdol)


Vitamin D stimulates the absorption of calcium and phosphate from the intestines and decreases bone resorption.

Vitamin E (Nutr-E-Sol)


Vitamin E protects polyunsaturated fatty acids in membranes from free radical injury and stabilizes RBC membranes. Nutr-E-Sol is a specially formulated vitamin E complexed with polyethylene glycol 1000 succinate to allow direct absorption without biliary emulsification. This is the formulation of choice for vitamin E–replacement therapy in patients with cholestasis. The formulation contains 400 IU vitamin E/15 mL.

Phytonadione (Mephyton)


Vitamin K-1 is necessary for the production of clotting factors II, VII, IX, and X by serving as a cofactor during carboxylation of glutamic acid residues.

Folic acid


Important cofactor for enzymes used in production of RBCs.


Trace Elements

Class Summary

These agents are inorganic substances found in small amounts in the tissues and are required for various metabolic processes.

Polysaccharide-iron complex (Niferex, Nu-Iron)


A nutritionally essential inorganic substance. Polysaccharide-iron complex is a product that contains ferric iron. 150 mg equals 150 mg of elemental iron. Also available as elixir containing 100 mg elemental iron per 5 mL.

Contributor Information and Disclosures

Pedro A de Alarcon, MD William H Albers Professor and Chair, Department of Pediatrics, University of Illinois College of Medicine at Peoria

Pedro A de Alarcon, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, American Society of Pediatric Hematology/Oncology, International Society of Hematology, International Society on Thrombosis and Haemostasis, Medical Society of the State of New York, New York Academy of Sciences, Society for Pediatric Research, Southern Society for Pediatric Research, Children's Oncology Group, Eastern Society for Pediatric Research, National Hemophilia Foundation, International Society for Experimental Hematology, Virginia Chapter of The American Academy of Pediatrics, Virginia Pediatric Society, American Federation for Clinical Research

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Steven K Bergstrom, MD Department of Pediatrics, Division of Hematology-Oncology, Kaiser Permanente Medical Center of Oakland

Steven K Bergstrom, MD is a member of the following medical societies: Alpha Omega Alpha, Children's Oncology Group, American Society of Clinical Oncology, International Society for Experimental Hematology, American Society of Hematology, American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA Executive Vice President, Chief Medical and Academic Officer, Renown Heath

Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American College of Healthcare Executives, American Society of Pediatric Hematology/Oncology, Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

J Martin Johnston, MD Associate Professor of Pediatrics, Mercer University School of Medicine; Director of Hematology/Oncology, The Children's Hospital at Memorial University Medical Center; Consulting Oncologist/Hematologist, St Damien's Pediatric Hospital

J Martin Johnston, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, International Society of Paediatric Oncology

Disclosure: Nothing to disclose.


Ulrike M Reiss, MD Associate Member, Department of Hematology, St Jude Children's Research Hospital

Disclosure: Nothing to disclose.

Mary E Ross, MD, PhD Assistant Professor, Department of Pediatrics, University of Illinois College of Medicine; Clinical Attending, St Jude Domestic Affiliate Clinic and Children’s Hospital of Illinois; Adjunct Faculty, St Jude Children’s Research Hospital

Disclosure: Nothing to disclose.

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This image (magnified X 2000) shows the spiculated thorny RBCs (acanthocytes) as observed in an individual with abetalipoproteinemia. These are indistinguishable from the acanthocytes shown in the next image, which are observed in an individual with spur cell hemolytic anemia. Used with permission from Little, Brown and Company.
This image (magnified X 2000) demonstrates acanthocytes in an individual with spur cell hemolytic anemia associated with alcoholic cirrhosis. Acanthocytes, unlike echinocytes or burr cells, have fewer spicules. Used with permission from Little, Brown and Company.
This image (magnified X 2000) shows echinocytes, or burr cells, a universal feature of uremia. The spicules of acanthocytes vary in length and width and project nonuniformly from the cell surface, while burr cells have regularly spaced, smoothly rounded crenulations. The second morphologic feature of RBCs in an individual with uremia is the presence of ellipsoid cells. Used with permission from Little, Brown and Company.
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