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

Int J Infect Dis. 2021 Feb:103:624-627. doi: 10.1016/j.ijid.2020.12.047. Epub 2021 Jan 4.


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.

Keywords: COVID-19; Hemophagocytic lymphohistiocytosis; Liver injury; SARS-CoV-2; Wilson’s disease.

Publication types

  • Case Reports

MeSH terms

  • COVID-19 / complications*
  • Diagnosis, Differential
  • Female
  • Hepatolenticular Degeneration / diagnosis*
  • Humans
  • Immunosuppression Therapy
  • Liver / virology*
  • Lymphohistiocytosis, Hemophagocytic / diagnosis
  • Lymphohistiocytosis, Hemophagocytic / etiology*
  • SARS-CoV-2*
  • Young Adult