What are hematologic symptoms of Wilson disease?

Updated: Feb 14, 2019
  • Author: Richard K Gilroy, MBBS, FRACP; Chief Editor: Praveen K Roy, MD, AGAF  more...
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Answer

Hemolytic anemia is a recognized, but rare (10%-15%), complication of the disease. Coombs-negative acute intravascular hemolysis most often occurs as a consequence of oxidative damage to the erythrocytes by the higher copper concentration. Any patient in whom acute hepatic failure occurs with a Coombs-negative intravascular hemolysis, modest elevations in serum aminotransferases, and a low serum alkaline phosphatase or ratio of alkaline phosphatase to bilirubin of less than 2 must be considered for a diagnosis of Wilson disease.


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