engleski

Association of acute intravascular hemolysis and acute liver failure as the initial manifestation of Wilson's disease

Acute intravascular hemolysis usually is an isolated transient manifestation of Wilson's disease, preceding the other symptoms by several years. In this paper, a patient in whom hemolysis was not an isolated manifestation, but was associated with severe acute liver failure, is described. In a 25-year-old female, the inicial manifestation of Wilson's disease was a syndrome in which acute intravascular hemolysis and acute liver failure were associated. This syndrome developed in three stages. The first stage was characterized by fatique, pain, fever and jaundice, the second by severe acute intravascular hemolysis, and the third by hepatic encelopathy with coma. The patient died from liver failure 20 days after the onset of the syndrome.

acute intravasculare hemolysis ; acute liver failure ; Wilson’s disease

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