Author:
Nakajima Koji,Hiejima Eitaro,Nihira Hiroshi,Kato Kentaro,Honda Yoshitaka,Izawa Kazushi,Kawabata Naoko,Kato Itaru,Ogawa Eri,Sonoda Mari,Okamoto Tatsuya,Okajima Hideaki,Yasumi Takahiro,Takita Junko
Abstract
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.
Subject
Immunology,Immunology and Allergy
Cited by
9 articles.
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