Acute hemolytic anemia as the first manifestation of Wilson's disease: report of two cases.

Introduction, - The liver and central nervous system are the usual targets of Wilson's disease, an inherited disorder of copper metabolism. Severe hem olytic anemia is an unusual complication of Wilson's disease. Exegesis, - We report two cases of Wilson's disease revealed by acute intra vascular hemolytic anemia associated with liver failure. Blood smear analys is showed stippled red cells in one case; hemolytic anemia improved within a few weeks in both patients but progressive liver failure required transpl antation in the other. Hemolysis probably results from the toxic effect of free serum copper on erythrocyte membrane. Conclusion, - Diagnosis of Wilson's disease must be considered in case of a cute hemolytic anemia associated with liver failure in young adults. (C) 20 01 Editions scientifiques et medicales Elsevier SAS.