title: Two unusual cases with Wilson's disease: hepatoma and fulminant hepatitis treated with plasma exchange.
abstract: We report two atypical cases of Wilson's disease. The first case is a 22-year-old male patient with a history of disease for 15 years and diagnosed as Wilson's disease upon investigations. Alpha-fetoprotein level was found elevated and computed tomography showed a 3.5-cm liver mass. Hepatocellular carcinoma was diagnosed. Radiofrequency ablation and liver transplantation were performed successfully. The second case is a 24-year-old female patient who presented with fulminant hepatitis. Urinary copper excretion and ceruloplasmin levels were suggestive of Wilson's disease. Despite chelation therapy, no improvement was observed. Plasma exchange therapy was performed for seven days. Her clinical status improved, and transplantation was no longer needed. To conclude, although hepatoma is rarely seen in Wilson's disease, patients should be examined regularly to diagnose it in a treatable stage. Removal of copper and toxic metabolites with plasma exchange therapy may be a way of treatment for fulminant hepatitis associated with Wilson's disease.
authors.affiliation: Gastroenterology Department, Hacettepe University Faculty of Medicine, Ankara, Turkey. maydinli@hacettepe.edu.tr
authors.forename: Musa
authors.initials: M
authors.lastname: Aydinli
authors.forename: Ozgur
authors.initials: O
authors.lastname: Harmanci
authors.forename: Osman
authors.initials: O
authors.lastname: Ersoy
authors.forename: Arzu T
authors.initials: AT
authors.lastname: Iskit
authors.forename: Osman
authors.initials: O
authors.lastname: Ozcebe
authors.forename: Osman
authors.initials: O
authors.lastname: Abbasoglu
authors.forename: Yusuf
authors.initials: Y
authors.lastname: Bayraktar
mesh_terms: D000328:Adult; D006528:Carcinoma, Hepatocellular / Q000209:etiology* / Q000628:therapy; D005260:Female; D006527:Hepatolenticular Degeneration / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D017114:Liver Failure, Acute / Q000209:etiology* / Q000628:therapy; D008113:Liver Neoplasms / Q000209:etiology* / Q000628:therapy; D016031:Liver Transplantation; D008297:Male; D010951:Plasma Exchange