Unusual Cause Of Severe Anemia In Cirrhosis

Abstract

Purpose: A 53 year old female with a history of alcohol abuse and cirrhosis presented with progressive leg swelling, fatigue and jaundice. She denied fevers, abdominal pain, melena, confusion, bloody stools or vomiting. Also, she has been sober for the past 8 months. The abnormal physical findings include tachycardia, scleral icterus, hepatomegaly, splenomegaly and 2+ bilateral leg edema. Initial labs showed critically low Hemoglobin 4 gm/dl, elevated indirect bilirubin 6.8 mg/dl and INR of 1.6. Later labs showed low haptoglobin <8 mg/dl [control 43-212], elevated LDH at 308 IU/L [control 98-192], elevated reticulocyte count of 6.2 [control 0.5-1.5], negative hepatitis panel, normal ceruloplasmin and a ferritin of 394. The peripheral smear revealed many burr cells. PRBC transfusion was started at ER. The EGD revealed portal hypertensive gastropathy but no signs of active or old bleeding were noticed. Therefore, the patient's anemia was consistent with the hemolytic process called Spur Cell Anemia seen secondary to End stage liver disease. The patient was treated with blood transfusions. When the hemoglobin was stable around 10 gm% for about 48 hrs she was discharged home. A couple of weeks later patient presented again with severe anemia [Hb 4.6 gm/dl] secondary to hemolysis. Again she was treated with blood transfusions. No GI source of bleeding found. Spur cell anemia is an unusual type of hemolytic anemia in patients with Liver Cirrhosis, particularly in those who abused alcohol. The mechanism of production of these cells has been attributed to the increased Cholesterol: Phospholipids ratio in RBC cell membranes with subsequent remodeling by the spleen because of rigid cell membranes. Therefore, these cells are more susceptible to hemolysis. The spur cell anemia should be suspected in any Cirrhotic patients presenting with hemolysis. The prognosis is extremely poor. They will need repeated blood transfusions and folate replacement. There is no effective treatment. Spur cell anemia may resolve after Liver transplant.Figure: Spur Cells in peripheral smear. [source: eMedicine with permisson from Dr Issam Makhoul]