Epstein-Barr Virus (EBV) related acute liver failure: A case series from the US Acute Liver Failure Study Group

Jessica L. Mellinger, Lorenzo Rossaro, Willscott E. Naugler, Satish N. Nadig, Henry Appelman, William M. Lee, Robert J. Fontana

Research output: Contribution to journalArticle

26 Citations (Scopus)

Abstract

Purpose: Acute liver failure (ALF) is a rare clinical syndrome associated with a high case fatality rate. Asymptomatic primary infection with Epstein-Barr virus (EBV) is common in the general population while acute hepatitis and jaundice are much less common and ALF has been rarely reported. We reviewed the presenting features as well as clinical outcomes amongst consecutive adults with EBV-related ALF. Methods: Amongst the 1,887 adult ALF patients enrolled into the US ALF Study Group from January 1998 to February 2012, there were four patients (0.21 %) with EBV-related ALF. Diagnostic criteria for acute EBV infection included compatible serologies and/or the detection of EBV-encoded RNA (EBER) in liver tissue. Results: Median patient age was 30 years (range 18-44); 75 % were male, and only 25 % were immunosuppressed. The median presenting ALT was 504 IU/mL (range 156-4,920), median Alk P was 431 (range 136-1,009), and median bilirubin was 17 mg/dL (range 13-22.1). Liver biopsy findings ranged from cholestasis to submassive necrosis with EBER + staining in two of the three samples tested. Although all of the patients were treated with an antiviral agent, two died of ALF, one underwent liver transplantation (LT) and one survived with supportive care and is well at 5 years. A review of the literature identified four additional LT recipients with favorable long-term outcomes. Conclusion: Primary EBV infection accounts for <1 % of consecutive adult ALF cases but is associated with a high case fatality rate. LT is associated with favorable short- and long-term outcomes.

Original languageEnglish (US)
Pages (from-to)1630-1637
Number of pages8
JournalDigestive diseases and sciences
Volume59
Issue number7
DOIs
StatePublished - 2014

Fingerprint

Acute Liver Failure
Human Herpesvirus 4
Liver Transplantation
Epstein-Barr Virus Infections
RNA
Asymptomatic Infections
Mortality
Liver
Cholestasis
Serology
Jaundice
Bilirubin
Hepatitis
Antiviral Agents
Necrosis
Staining and Labeling
Biopsy

Keywords

  • Acyclovir
  • Fulminant hepatic failure
  • Liver transplantation
  • Viral hepatitis

ASJC Scopus subject areas

  • Gastroenterology
  • Physiology

Cite this

Epstein-Barr Virus (EBV) related acute liver failure : A case series from the US Acute Liver Failure Study Group. / Mellinger, Jessica L.; Rossaro, Lorenzo; Naugler, Willscott E.; Nadig, Satish N.; Appelman, Henry; Lee, William M.; Fontana, Robert J.

In: Digestive diseases and sciences, Vol. 59, No. 7, 2014, p. 1630-1637.

Research output: Contribution to journalArticle

Mellinger, Jessica L. ; Rossaro, Lorenzo ; Naugler, Willscott E. ; Nadig, Satish N. ; Appelman, Henry ; Lee, William M. ; Fontana, Robert J. / Epstein-Barr Virus (EBV) related acute liver failure : A case series from the US Acute Liver Failure Study Group. In: Digestive diseases and sciences. 2014 ; Vol. 59, No. 7. pp. 1630-1637.
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AU - Rossaro, Lorenzo

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AU - Nadig, Satish N.

AU - Appelman, Henry

AU - Lee, William M.

AU - Fontana, Robert J.

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N2 - Purpose: Acute liver failure (ALF) is a rare clinical syndrome associated with a high case fatality rate. Asymptomatic primary infection with Epstein-Barr virus (EBV) is common in the general population while acute hepatitis and jaundice are much less common and ALF has been rarely reported. We reviewed the presenting features as well as clinical outcomes amongst consecutive adults with EBV-related ALF. Methods: Amongst the 1,887 adult ALF patients enrolled into the US ALF Study Group from January 1998 to February 2012, there were four patients (0.21 %) with EBV-related ALF. Diagnostic criteria for acute EBV infection included compatible serologies and/or the detection of EBV-encoded RNA (EBER) in liver tissue. Results: Median patient age was 30 years (range 18-44); 75 % were male, and only 25 % were immunosuppressed. The median presenting ALT was 504 IU/mL (range 156-4,920), median Alk P was 431 (range 136-1,009), and median bilirubin was 17 mg/dL (range 13-22.1). Liver biopsy findings ranged from cholestasis to submassive necrosis with EBER + staining in two of the three samples tested. Although all of the patients were treated with an antiviral agent, two died of ALF, one underwent liver transplantation (LT) and one survived with supportive care and is well at 5 years. A review of the literature identified four additional LT recipients with favorable long-term outcomes. Conclusion: Primary EBV infection accounts for <1 % of consecutive adult ALF cases but is associated with a high case fatality rate. LT is associated with favorable short- and long-term outcomes.

AB - Purpose: Acute liver failure (ALF) is a rare clinical syndrome associated with a high case fatality rate. Asymptomatic primary infection with Epstein-Barr virus (EBV) is common in the general population while acute hepatitis and jaundice are much less common and ALF has been rarely reported. We reviewed the presenting features as well as clinical outcomes amongst consecutive adults with EBV-related ALF. Methods: Amongst the 1,887 adult ALF patients enrolled into the US ALF Study Group from January 1998 to February 2012, there were four patients (0.21 %) with EBV-related ALF. Diagnostic criteria for acute EBV infection included compatible serologies and/or the detection of EBV-encoded RNA (EBER) in liver tissue. Results: Median patient age was 30 years (range 18-44); 75 % were male, and only 25 % were immunosuppressed. The median presenting ALT was 504 IU/mL (range 156-4,920), median Alk P was 431 (range 136-1,009), and median bilirubin was 17 mg/dL (range 13-22.1). Liver biopsy findings ranged from cholestasis to submassive necrosis with EBER + staining in two of the three samples tested. Although all of the patients were treated with an antiviral agent, two died of ALF, one underwent liver transplantation (LT) and one survived with supportive care and is well at 5 years. A review of the literature identified four additional LT recipients with favorable long-term outcomes. Conclusion: Primary EBV infection accounts for <1 % of consecutive adult ALF cases but is associated with a high case fatality rate. LT is associated with favorable short- and long-term outcomes.

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