Frontiers in Immunology (Jan 2022)

Case Report: A Case of Epstein-Barr Virus-Associated Acute Liver Failure Requiring Hematopoietic Cell Transplantation After Emergent Liver Transplantation

  • Koji Nakajima,
  • Eitaro Hiejima,
  • Hiroshi Nihira,
  • Kentaro Kato,
  • Yoshitaka Honda,
  • Kazushi Izawa,
  • Naoko Kawabata,
  • Itaru Kato,
  • Eri Ogawa,
  • Mari Sonoda,
  • Tatsuya Okamoto,
  • Hideaki Okajima,
  • Takahiro Yasumi,
  • Junko Takita

DOI
https://doi.org/10.3389/fimmu.2022.825806
Journal volume & issue
Vol. 13

Abstract

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Hepatic manifestations of Epstein-Barr virus (EBV) infection are relatively common, mild, and self-limiting. Although fulminant hepatic failure has been reported in a few cases, the contributing factors are unclear. This report discusses a pediatric case of EBV-associated acute liver failure that required urgent liver transplantation; however, liver damage continued to progress post-liver replacement. Monoclonal CD8+ T cells that preferentially infiltrated the native and transplanted liver were positive for EBV-encoded small RNA, suggesting a pathophysiology similar to that of EBV-associated hemophagocytic lymphohistiocytosis and chronic active EBV infection. Therefore, subsequent chemotherapy and hematopoietic cell transplantation was conducted, which led to cure. This is the first case of EBV-associated acute liver failure that relapsed post-liver transplant. As such, it sheds light on an under-recognized clinical entity: liver-restricted hyperinflammation caused by EBV-infected monoclonal CD8+ T cells. This phenomenon needs to be recognized and differentiated from hepatitis/hepatic failure caused by EBV-infected B cells, which has a relatively benign clinical course.

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