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Spur Cell Anemia Clinical Presentation

  • Author: Christopher D Braden, DO; Chief Editor: Emmanuel C Besa, MD  more...
 
Updated: Dec 06, 2015
 

History

The symptoms of spur cell anemia are related to the anemia and to the underlying disease.

Spur cell anemia in severe liver disease

In spur cell anemia, the hemoglobin level usually falls to less than 10 g/dL and, occasionally, to levels as low as 5 g/dL. This fall may be associated with severe jaundice and rapid deterioration of liver function, coagulopathy, and hepatic encephalopathy.

In its chronic presentation, the anemia accompanying the alcoholic cirrhosis is mild, whereas in the acute presentation, the anemia develops weeks to months before death and as liver function deteriorates.

The course of spur cell anemia correlates with the liver function. Cases of reversal of the hemolytic anemia have been reported after improvement of liver disease.

Spur cell anemia has been reported in cases of pediatric cholestatic liver disease.[6] In most cases, the condition is transient and resolves with the improvement of underlying liver disease.

Hemosiderosis is reported in 20% of patients undergoing orthotopic liver transplantation for alcoholic liver disease. Spur cell hemolytic anemia is present in 75% of these patients. In the absence of the C282Y/HFE hemochromatosis gene mutation, spur cell hemolytic anemia is postulated to be responsible for the hemosiderosis related to repeated blood transfusions and increasing intestinal iron absorption.

Acanthocytosis in abetalipoproteinemia

The clinical presentation of acanthocytosis in cases of abetalipoproteinemia includes ataxia, RP that may lead to blindness, and fat malabsorption. Symptoms related to the deficiency of lipid-soluble vitamins (ie, A, K, E, D) may be seen. Spur cells (50-90%) are present on the peripheral smear, and the hemolysis and anemia are mild.

Abetalipoproteinemia is an autosomal-recessive disease that manifests in the first months of life, with steatorrhea, abdominal distention, and growth retardation. Neurologic symptoms appear in patients aged 5-10 years and may progress to death in the second or third decade.

Chorea-acanthocytosis syndrome

The median age at onset of symptoms in chorea-acanthocytosis syndrome is 32 years. Median survival is 8-14 years. Limb chorea is the initial symptom in many cases, but, because it may be mild, patients may be able to suppress it for long periods before the other symptoms are evident.

Orofacial tics, buccolingual dyskinesia, and tongue biting that causes major problems with eating and swallowing occur early in the disease course. Neurogenic muscle hypotonia, atrophy, and areflexia are common. Dysarthria develops during the course of the disease and occasionally may be the presenting feature.

Dementia and seizures are relatively common. Organic personality changes with impulsive, easily distracted behavior occur. Apathy and loss of insight are the most consistent symptoms. Other psychiatric symptoms that are encountered include depression, anxiety, paranoid delusions, and obsessive-compulsive features.

The percentage of acanthocytes in the peripheral blood varies from 20-50%. Patients do not have anemia.

McLeod phenotype

This condition is characterized by a mild, compensated hemolytic anemia and, occasionally, late-onset myopathy or chorea.

The acanthocyte number varies between 25% and 85%, and serum creatine kinase (CK) is elevated. This disorder is also described in association with CGD, RP, and DMD. The deletion of band Xp21 affects all or some of the genetic loci of these disorders because of their close proximity on the short arm of chromosome X.

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Physical Examination

In advanced liver disease, jaundice, hepatosplenomegaly, ascites, altered mental status, and bleeding diathesis may be present. In abetalipoproteinemia, ataxia and decreased visual acuity are the main findings.

Chorea-acanthocytosis syndrome is characterized by limb chorea, orofacial dyskinesia, muscle atonia, and atrophy.

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Contributor Information and Disclosures
Author

Christopher D Braden, DO Hematologist/Oncologist, Chancellor Center for Oncology at Deaconess Hospital

Disclosure: Nothing to disclose.

Coauthor(s)

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, American Society of Hematology

Disclosure: Nothing to disclose.

Mansoor Javeed, MD, FACP Clinical Assistant Professor of Medicine, University of California, Davis, School of Medicine; Consultant, Sierra Hematology-Oncology Medical Center

Mansoor Javeed, MD, FACP is a member of the following medical societies: American College of Physicians, Pennsylvania Medical Society

Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD Professor Emeritus, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American Society of Clinical Oncology, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, New York Academy of Sciences

Disclosure: Nothing to disclose.

Acknowledgements

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, and American Society of Hematology

Disclosure: Nothing to disclose.

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: Nothing to disclose.

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.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

References
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  9. Kok VC, Lee CK, Horng JT, Lin CC, Sung FC. Reappraisal of the etiology of extracorpuscular non-autoimmune acquired hemolytic anemia in 2657 hospitalized patients with non-neoplastic disease. Clin Med Insights Pathol. 2014. 7:11-4. [Medline].

  10. Marks PW. Hematologic manifestations of liver disease. Semin Hematol. 2013 Jul. 50(3):216-21. [Medline].

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

  12. Redman CM, Russo D, Lee S. Kell, Kx and the McLeod syndrome. Baillieres Best Pract Res Clin Haematol. 1999 Dec. 12(4):621-35. [Medline].

  13. 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. 1999 Mar. 101(1):25-31. [Medline].

  14. 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. 1998 Apr 15. 65(7):993-5. [Medline].

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