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.

Liver transplantation for children with Wilson disease: comparison of outcomes between children and adults

“…This mechanism explains the relatively high rate of hemodialysis before LT. Some of the modalities to protect the kidneys include plasmapheresis (15, 16), fractionated plasma separation and adsorption (FPSA) system (17), or molecular adsorbents recirculating system (MARS) that remove the toxic free copper from the serum (18).…”

Successful treatment of fulminant Wilson's disease without liver transplantation

“…To the best of our knowledge, four fulminant WD patients with RWPI ≥11 avoided LTx. We summarize the clinical features, RWPI, major laboratory findings, therapeutic modality, and outcomes of five patients including the present one (Table ) . The onset age ranged from 10 to 19 years (mean, 15.3 years).…”

Wilson’s disease: A review of what we have learned

“…As bridging therapies to liver transplantation, or as an alternative altogether in regions were liver transplantation is not possible, strategies such as rapid plasma exchange [124,125] via any method such as plasmapheresis [126] , hemofiltration [127,128] , albumin dyalisis [129] , or exchange transfusion, may be successfully used to lower circulating copper levels, renal protection from coppermediated tubular damage, and reduce hemolysis [130] . Albeit some degree of improvement has been reported, the need for liver transplantation has not been obviated in numerous cases, although successful treatment without transplantation has been reported [131] . The molecular adsorbent recycling system ultrafiltration device, which combines ion exchange with albumin dialysis might provide some therapeutic efficacy in this setting, achieving copper removal and clinical stabilization, that might constitute a bridge for liver transplantation [132][133][134][135] .…”