What Is Spur Cell Anemia?
Spur cell anemia is an extrinsic (acquired) hemolytic anemia that is associated with chronic liver disease. It is characterized by the presence of an increased number of large erythrocytes, which are covered with rough or thorny projections usually varying in thickness, length, and distribution. These red blood cells are called acanthocytes. Spur cell anemia can be considered an independent risk factor of mortality in chronic liver cirrhosis cases and is usually associated with a very poor prognosis. Liver transplantation is the most effective treatment for spur cell anemia. The first case of the disorder was reported in 1964.
What Are the Causes of Spur Cell Anemia?
The causes of anemia related to liver disease may be
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Gastrointestinal Bleeding
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Enlarged veins of the esophagus.
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Dilated gastric blood vessels.
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Gastric antral vascular ectasia (a condition where the blood vessels lining the stomach are easily prone to rupture, causing frequent bleeding.
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Low blood platelet counts in the blood.
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Clotting disorder.
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Hemolysis
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An overactive spleen (hypersplenism) is secondary to portal vein hypertension.
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Hemolytic anemia of the autoimmune type is usually related to autoimmune hepatitis.
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Wilson’s disease with increased copper accumulation in tissues.
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Excessive Alcohol Consumption- Alcohol, if consumed in excessive amounts, can be a cause of spur cell anemia.
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Bone Marrow Toxicity- The toxicity of the bone marrow can cause spur cell anemia.
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Nutritional Deficiency- Vitamin B12 and Folic acid deficiency can also be one of the causes of this type of anemia.
What Are the Clinical Features of Spur Cell Anemia?
The clinical manifestations of the disorder include jaundice, edema of the lower extremities (lower limb swelling), dyspnea (difficulty in breathing), difficulty in walking, conjunctival icterus (yellowish discoloration of sclera due to bilirubin deposition), mild distention of the abdomen, and bilateral pitting edema (an indentation is created when pressure is created in the area of fluid accumulation), with scattered bruising.
The hemoglobin level usually falls below 0.02 pound per deciliter, and occasionally the levels reach as low as 0.01 pound per deciliter in spur cell anemia. The reduction in hemoglobin levels may be related to severe jaundice and rapid liver failure, liver function deterioration, coagulopathy (excessive bleeding or clotting), and hepatic encephalopathy (nervous system disorder caused due to chronic liver disease).
How Is the Diagnosis of Spur Cell Anemia Established?
It is important to establish the diagnosis of spur cell anemia. Laboratory tests, liver biopsy, peripheral blood smear, and excluding other chronic liver diseases are useful in determining the disorder.
Laboratory tests usually reveal the following:
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Acute anemia, with a rapid fall in the hemoglobin level from 10.6 grams per deciliter to 6.3 grams per deciliter in a period of two weeks. The normal hemoglobin value ranges from 13.0–17.0.
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Macrocytosis (larger than normal red blood cells), with an increased mean corpuscular volume above 100 cubic microns.
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Increased thickness of the red blood cells with an overall width of more than 22% (normal range is 11%–16%).
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Increased reticulocyte count of more than 9% (0.4%–2.0%).
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Increased lactate dehydrogenase levels of more than 412 units per liter. The normal level ranges between 140 to 280 units per liter.
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Thrombocytopenia is observed.
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Hyperbilirubinemia (increased serum bilirubin levels) will be noted.
Computed tomography usually reveals only diffuse swelling and mild ascites (fluid accumulation in the abdomen) without hemorrhage.
A peripheral blood smear will usually reveal various irregularly shaped red blood cells with spike-like or thorn-like projections. These cells are also known as acanthocytes or spur cells. An increased number of reticulocytes are also seen on the peripheral blood smear, thereby establishing the diagnosis of spur cell anemia.
On performing a liver biopsy, typical hepatocellular ballooning and Mallory bodies (intracytoplasmic hyaline inclusions of liver cells) are seen on microscopic examination. These histopathological findings are suggestive of liver cirrhosis.
What Is the Treatment and Management of Spur Cell Anemia?
The management is usually supportive for spur cell anemia patients.
Spur-cell anemia is commonly associated with a very poor prognosis. Liver transplantation is considered the only definitive treatment therapy. In addition, the transfusion of blood to the patient is also considered a part of the treatment regimen to treat symptomatic anemia. Besides, the patient suffering from spur cell anemia should also be supplemented with branched-chain amino acids, levocarnitine to increase energy production, certain antibiotics, zinc, folic acid, thiamin (vitamin B1), pyridoxine (vitamin B6), cobalamin (vitamin B12), ascorbic acid (vitamin C), and omega fatty acids.
Supportive treatment therapy also includes blood transfusion and discontinuation of the consumption of alcohol. Blood transfusion is essential to increase the oxygenation of the blood and increase the blood hemoglobin levels. Bone marrow-suppressive pharmacological drugs should also be discontinued. Respiratory care with intubation is also recommended in the affected individuals. The doctor should suggest appropriate therapeutic measures to eliminate the chief cause of chronic liver disease. Further research is required to establish the treatment strategy with corticosteroids. Patients who did not go for liver transplantation usually show poor prognosis. However, if the cases of spur cell anemia are recurrent even after liver transplantation, then it could be due to graft failure. Management of spur cell anemia is very important and crucial for improving the prognosis of liver cirrhosis patients.
What Is the Prognosis of Spur Cell Anemia?
Spur cell anemia has got a poor prognosis. It is important to seek medical advice as soon as possible. Liver transplantation is a definitive treatment plan.
Conclusion:
Spur cell anemia is a type of anemia that is related to cirrhosis of the liver. It is a type of acquired hemolytic anemia. It is also characterized by an increase in the number of large red blood cells. Spur cell anemia is a very serious and life-threatening condition in patients suffering from liver cirrhosis. If left untreated, it can lead to fatal results. It is very important to treat spur cell anemia in liver cirrhosis patients, or else it can lead to extremely poor prognosis in such patients. Liver transplantation is the mainstay of treatment in these patients. However, more research should be carried out in the therapeutic field to improve the overall living condition of the affected patient.
