A 15-year-old girl with severe hemolytic Wilson's crisis recovered without transplantation after extracorporeal circulation with the Prometheus system.
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.
Aagaard
Niels Kristian
NK
Department of Medicine V (Hepatology and Gastroenterology), Aarhus University Hospital, NBG, Aarhus, Denmark. nielaaga@rm.dk
Thomsen
Karen Louise
KL
Holland-Fischer
Peter
P
Jørgensen
Søren Peter
SP
Ott
Peter
P
D000111:Acetylcysteine / Q000627:therapeutic use; D000293:Adolescent; D000931:Antidotes / Q000627:therapeutic use; D003300:Copper / Q000302:isolation & purification; D005112:Extracorporeal Circulation / Q000295:instrumentation*; D005260:Female; D016166:Free Radical Scavengers / Q000627:therapeutic use; D006461:Hemolysis; D006527:Hepatolenticular Degeneration / Q000097:blood* / Q000175:diagnosis / Q000628:therapy*; D006801:Humans; D008099:Liver / Q000503:physiopathology*; D010396:Penicillamine / Q000627:therapeutic use; D012709:Serum Albumin / Q000378:metabolism; D012720:Severity of Illness Index
D000931:Antidotes; D016166:Free Radical Scavengers; D012709:Serum Albumin; D003300:Copper; D010396:Penicillamine; D000111:Acetylcysteine