International Journal of Infectious Diseases (2021-02-01)

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

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

Journal volume & issue
Vol. 103
pp. 624 – 627


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A 21-year-old woman was hospitalized due to coronavirus disease 2019 (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 a hepatic severe acute respiratory syndrome coronavirus 2 (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. In the differential diagnosis, an 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.