Hepatobiliary and pancreatic: Spur cell anemia associated with alcoholic cirrhosis

A 47-year-old man was admitted to hospital with jaundice and ascites. Cirrhosis was attributed to alcohol abuse as his alcohol intake had been high for at least 10 years. Prior to admission, he had ceased drinking alcohol and had been treated with diuretics and pentoxifylline. Blood tests revealed an elevated plasma bilirubin (11 mg/dL or 187 mmol/l), a mild elevation of alkaline phosphatase (128 u/l) and normal levels of alanine aminotransferase and aspartate aminotransferase. His plasma albumin was marginally low (31 g/l) and he had a prolonged prothrombin time (22.8 seconds). His hemoglobin was 73 g/l and there were acanthocytes or spur cells on a peripheral blood smear (Figure 1). Features consistent with hemolysis included indirect hyperbilirubinemia, hemoglobinuria and a raised serum level of lactate dehydrogenase (588 u/l). A direct Coomb’s test was negative while a bone marrow biopsy revealed a hypercellular smear. There were elevated serum levels of vitamin B12, folic acid and ferritin and low serum levels of lipids. After 4 months, his general condition deteriorated with the development of hepatic encephalopathy and recurrent episodes of spontaneous bacterial peritonitis. He required blood transfusions at intervals of about 2 weeks because of persistent anemia. A liver biopsy revealed cirrhosis with significant iron overload (grade IV). He has been started on iron chelation therapy and is currently on a waiting list for liver transplantation. Spur cells or acanthocytes are large erythrocytes with spike-like projections that vary in width, length and distribution. Spur cell anemia is characterized by non-immune hemolysis with a shortened red cell survival time and secondary iron overload. Most patients have advanced alcoholic cirrhosis or alcoholic hepatitis with a poor prognosis. However, spur cell anemia does resolve after liver transplantation. The pathogenesis of spur cell anemia is likely to involve changes in serum lipids that affect the lipid composition and fluidity of erythrocyte membranes. In order to support the hypothesis of a plasma factor, we incubated red blood cells from a control subject with plasma from our patient for 18 hrs at 37°C. The red cells showed a marked increase in spur cell formation (Figure 2) when compared to cells incubated in control plasma.