A 15-Year-Old Girl with Severe Hemolytic Wilson’s Crisis Recovered without Transplantation after Extracorporeal Circulation with the Prometheus® System

Abstract

Background: Wilson’s disease (WD) can present in a fulminant form with hepatocellular dysfunction, hemolysis and multiorgan failure (Wilson’s crisis). We present a previously healthy young woman with severe WD whose WD severity score was 13. A score >11 indicates a poor chance of survival and liver transplantation will usually be recommended. Methods: Penicillamine and acetylcysteine were initially administered, but the patient deteriorated further, and extracorporeal liver support with the Prometheus® FPSA (fractionated plasma separation and adsorption) system was initiated. The patient was treated 6 h daily during 3 consecutive days. Results: Severe hemolysis was reduced to low-grade hemolysis, with no further need for transfusions. The mental state improved and after 4 months practically all biochemical markers were normalized. Conclusions: This is the first report of FPSA albumin dialysis of a patient with Wilson’s crisis and the first report in which a patient with a WD score >11 survived without transplantation.