A New Wilson Index (NWI)11 has been used as a predictor of mortality without transplantation in fulminant Wilson illness since 2005. (WD). Plasma exchange is being promoted as a novel therapeutic option. This study describes a case of a patient with fulminant WD who was treated with plasma exchange. All reported instances using plasma exchange for fulminant WD were thoroughly examined. A 14-year-old girl was admitted to the hospital with hemolysis and fulminant liver failure. She had no encephalopathy and a NWI of 14. Plasma exchange was initiated immediately as a bridge to transplantation. Plasma exchange and later chelation treatment with d-penicillamine resulted in complete remission. She has now been transplant-free for three years. A study of the literature revealed 37 individuals with fulminant WD and NWI 11 who were treated with plasma exchange. Seventeen of these individuals (46 percent) healed without the need for a transplant.
Despite a NWI 11, several case reports and case series show transplant-free survival after plasma exchange and subsequent chelation treatment. Plasma exchange has an effect on the clinical course and is a treatment option in adolescents and young adults with fulminant WD.
