Secondary hemophagocytic lymphohistiocytosis and severe liver injury induced by hepatic SARS-CoV-2 infection unmasking Wilson’s disease: balancing immunosuppression

Lubnow, Matthias; Schmidt, Barbara; Fleck, Martin; Salzberger, Bernd; Müller, Thomas; Peschel, Georg; Schneckenpointner, Roland; Lange, Tobias; Hitzenbichler, Florian; Kieninger, Martin; Lunz, Dirk; Graf, Bernhard; Brochhausen, Christoph; Weber, Florian; Lüke, Florian; Peterhoff, David; Schuster, Philipp; Hiergeist, Andreas; Offner, Robert; Hehr, Ute; Wallner, Stefan; Hanses, Frank; Schmid, Stephan; Weigand, Kilian; Geismann, Florian; Poeck, Hendrik; Pukrop, Tobias; Evert, Matthias; Gessner, Andre; Burkhardt, Ralph; Herr, Wolfgang; Maier, Lars S.; Heudobler, Daniel

A 21 year old woman was hospitalized due to Covid-19 associated respiratory and hepatic impairment concomitant with severe hemolytic anemia. Upon diagnosis of secondary hemophagocytic lymphohistiocytosis immunosuppression with anakinra and steroids was started, leading to hepatic SARS-CoV-2 infection and viremia. Subsequent liver biopsy revealed virus particles in hepatocytes by electron microscopy and SARS-CoV-2 virus could be isolated and cultured. Immunosuppression was stopped and convalescent donor plasma given. As differential diagnosis acute crisis of Wilson’s disease was raised by laboratory and genetic testing. This case highlights the complexity of balancing immunosuppression to control hyperinflammation versus systemic SARS-CoV-2 dissemination.