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

Frontiers in Immunology

Hideaki Okajima¹  Tatsuya Okamoto²  Eri Ogawa²  Mari Sonoda²  Junko Takita³  Kazushi Izawa³  Eitaro Hiejima³  Kentaro Kato³  Naoko Kawabata³  Itaru Kato³  Hiroshi Nihira³  Takahiro Yasumi³  Koji Nakajima³  Yoshitaka Honda³ 

[1] Department of Pediatric Surgery, Kanazawa Medical University, Ishikawa, Japan;Department of Pediatric Surgery, Kyoto University Hospital, Kyoto, Japan;Department of Pediatrics, Kyoto University Hospital, Kyoto, Japan;

关键词: hemophagocytic lymphohistiocytosis (HLH);    Epstein-Barr virus (EBV);    acute liver failure (ALF);    hematopoietic cell transplantation (HCT);    case report;    CAEBV infection;   

DOI  :  10.3389/fimmu.2022.825806

来源: DOAJ

【 摘 要 】

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.

【 授权许可】

Unknown