PMC8131166_01
Liver biopsy specimen. (a) Annular fibrosis (AF) surrounding hepatocyte nodules (Masson trichrome staining, original magnification x 50). (b) Chronic hepatitis with lobular activity (open arrow), ballooned hepatocytes (black arrows), and mild steatosis (H&E staining, original magnification x 50). (c) Black arrows indicate Victoria blue staining deposits in periportal areas.
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fig1
Increased hepatocyte copper-associated protein deposition was observed in periportal areas using Victoria blue stain (Figure 1).
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.
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fig2
However, the following days were associated with a rapid clinical deterioration and majoration of hemolytic anemia with low hemoglobin levels in the range of 60 to 70 gr/l, necessitating repeated blood transfusions (Figure 2). The patient rapidly recovered from hemolysis, as no further blood transfusions were necessary after day 7 (see Figure 2).
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.
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fig3
A total of 9 MARS dialysis sessions (each session lasting 6-8 hours) were administered starting from day 6 to day 17, with an excellent clinical tolerance, no bleeding complications, and only a marginal decrease in platelet count.  Figure 3 depicts the MARS device and the dark-brown-colored adsorber cartridge filled with copper.
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].
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fig4
The evolution of WD prognostic index during the hospitalization is depicted in Figure 4.