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

ABSTRACT: 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.

SUBMITTER: Lubnow M 

PROVIDER: S-EPMC7781512 | biostudies-literature | 2021 Jan

REPOSITORIES: biostudies-literature

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

Lubnow Matthias M   Schmidt Barbara B   Fleck Martin M   Salzberger Bernd B   Müller Thomas T   Peschel Georg G   Schneckenpointner Roland R   Lange Tobias T   Hitzenbichler Florian F   Kieninger Martin M   Lunz Dirk D   Graf Bernhard B   Brochhausen Christoph C   Weber Florian F   Lüke Florian F   Peterhoff David D   Schuster Philipp P   Hiergeist Andreas A   Offner Robert R   Hehr Ute U   Wallner Stefan S   Hanses Frank F   Schmid Stephan S   Weigand Kilian K   Geismann Florian F   Poeck Hendrik H   Pukrop Tobias T   Evert Matthias M   Gessner Andre A   Burkhardt Ralph R   Herr Wolfgang W   Maier Lars S LS   Heudobler Daniel D  

International journal of infectious diseases : IJID : official publication of the International Society for Infectious Diseases 20210104

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 vir  ...[more]

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