Journal
REVUE DE MEDECINE INTERNE
Volume 22, Issue 3, Pages 280-283Publisher
ELSEVIER FRANCE-EDITIONS SCIENTIFIQUES MEDICALES ELSEVIER
DOI: 10.1016/S0248-8663(00)00329-5
Keywords
Wilson's disease; haemolytic anemia; stippled red cells
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Introduction, - The liver and central nervous system are the usual targets of Wilson's disease, an inherited disorder of copper metabolism. Severe hemolytic anemia is an unusual complication of Wilson's disease. Exegesis, - We report two cases of Wilson's disease revealed by acute intravascular hemolytic anemia associated with liver failure. Blood smear analysis showed stippled red cells in one case; hemolytic anemia improved within a few weeks in both patients but progressive liver failure required transplantation 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 acute hemolytic anemia associated with liver failure in young adults. (C) 2001 Editions scientifiques et medicales Elsevier SAS.
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