Workup
Approach Considerations
Findings on the workup for spur cell anemia include the following:
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Complete blood cell count - Variable degrees of anemia are present, with the hematocrit commonly between 15% and 20%. White blood cell and platelet counts may be normal; however, they are decreased in most cases due to severe, advanced liver disease.
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Reticulocyte count - An increase in the reticulocyte count depends on the degree of the anemia, but the count is usually greater than 5%. In certain cases, the reticulocyte count may be decreased as a result of concomitant folate deficiency.
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Liver function tests - Hyperbilirubinemia, predominantly indirect bilirubin, is present, and its increase parallels the hemolysis. Synthetic liver function is decreased, as evidenced by low levels of albumin and fibrinogen and prolongation of the prothrombin time (PT) and activated partial thromboplastin time (aPTT).
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Plasma lipids - This study helps screen suspected cases of abetalipoproteinemia. These patients have very low levels of serum cholesterol, phospholipid, and triglyceride. Lipoprotein electrophoresis reveals the absence of beta-lipoproteins.
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Blood typing - Kell antisera react poorly with red blood cells, white blood cells, or both in the McLeod phenotype.
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Serum creatine kinase - In McLeod syndrome, the creatine kinase levels are increased.
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Erythrocyte sedimentation rate (ESR) - In patients with neuroacanthocytosis, particularly McLeod syndrome and chorea acanthocytosis, ESR tends to be low. It decreases as the fraction of acanthocytes increases. [20]
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Intestinal biopsy in abetalipoproteinemia - This procedure reveals the presence of fat droplets within the mucosal cells.
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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 that 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, particularly if obstructive jaundice is present, the smear may also reveal target cells (ie, erythrocytes in which hemoglobin is concentrated in the center and on the periphery, with a colorless zone in between, creating a "bullseye" appearance; see the image below)
Acanthocytes with target cells in a patient with advanced liver disease.
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Acanthocytes with target cells in a patient with advanced liver disease.
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