ALCOHOL-INDUCED BURR CELL (ECHINOCYTIC) HEMOLYTIC-ANEMIA AND HEMOCHROMATOSIS

A 51-year-old man with chronic alcoholic liver disease developed a sev ere haemolytic anaemia characterized by the presence of circulating bu rr-shaped cells (echinocytes). Several transfusions of packed red cell s were ineffective in raising the haemoglobin concentration, showing t hat the abnormality was acquired by the transfused cells. Liver biopsi es revealed haemochromatosis. Haematological parameters normalized fou r months after the patient stopped drinking alcohol, but burr cells we re still present and erythrocyte life-span was still markedly shortene d at one year follow-up. Since serum cholesterol, HDL-cholesterol, and Apo-AI and Apo-B lipoproteins were considerably decreased, the lipid composition of the red cell membrane was studied. Findings showed that echinocytosis occurred with no change in membrane cholesterol content , nor in cholesterol:phospholipid ratio, but with an alteration in the phosphatidylserine and phosphatidylinositol concentrations. While hae mochromatosis was most likely the cause of the erythrocyte anomaly, al cohol intake was probably responsible for the acute onset of haemolyti c anaemia with effects directly on the erythrocyte membrane as well as mediated by the progressive hepatic injury, with alterations in the p lasma and successively in the intramembrane lipid composition.