Management of Acute Wilsonian Hepatitis with Severe Hemolysis: A Successful Combination of Chelation and MARS Dialysis

Abstract

Wilson's disease is a rare hereditary disorder of copper metabolism leading to progressive accumulation of copper in several organs including the brain and the liver. Acute liver failure is a relatively rare hepatic manifestation of WD which may require urgent liver transplantation if medical treatment fails. We report here the case of a young woman who presented with classic acute Wilsonian hepatitis complicated by liver and renal failure and a severe hemolysis related to massive nonceruloplasmin bound copper accumulation requiring repeated blood transfusions. The early initiation of a combined treatment including conventional chelation therapy and repeated MARS dialysis sessions allowed a rapid control of hemolysis, a progressive decrease of free copper overload, and clinical recompensation without liver transplantation.

Figure 1

Liver biopsy specimen. (a) Annular fibrosis (AF) surrounding hepatocyte nodules (Masson trichrome staining, original magnification × 50). (b) Chronic hepatitis with lobular activity (open arrow), ballooned hepatocytes (black arrows), and mild steatosis (H&E staining, original magnification × 50). (c) Black arrows indicate Victoria blue staining deposits in periportal areas.

Figure 2

Graphical illustration of the patient's course during hospitalization. Each cartoon drop indicates a blood transfusion. The violet line indicates INR, and the red dotted line corresponds to hemoglobin level. The orange line and the green solid line illustrate serum creatinine and total bilirubin values, respectively. Black triangles correspond to the individual MARS dialysis session.

Figure 3

Illustration of the MARS device prior to use (a) and at the end of the initial session (b). The initially colorless diaMARS IE250 adsorber cartridge (1) became dark brown at the end of the procedure due to massive copper deposits (2). (c) A cartridge from a historical case of alcoholic cirrhosis and hepatorenal syndrome under MARS therapy showing, instead, a yellowish brown color due to bilirubin pigments.

Figure 4

Evolution of Wilson's disease prognostic index during hospitalization. A score ≥ 11 (dashed line) is associated with a high probability of death without LT according to [8].