La Revue de medecine interne · 2001
[Hemolytic anemia disclosing Wilson's disease. Report of 2 cases].
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Michel M, Lafaurie M, Noël V, Pico L, Bastie A, Godeau B, Schaeffer A
Аннотация
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.
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.