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Epstein-Barr virus-induced sickle hepatopathy.

Alison S Towerman1,2, David B Wilson1,3, Monica L Hulbert1

  • 1Departments of Pediatrics, Washington University School of Medicine, St. Louis Children's Hospital, St. Louis, Missouri, USA.

Pediatric Blood & Cancer
|June 28, 2021
PubMed
Summary

Epstein-Barr virus (EBV) infection can trigger acute sickle hepatopathy in patients with hemoglobin SC disease. This can manifest as hepatic sequestration or severe cholestasis, highlighting a new association for this condition.

Keywords:
cholestasishemoglobin SC diseasehepatic sequestrationinfectious mononucleosissickle cell disease

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Area of Science:

  • Hematology
  • Hepatology
  • Infectious Diseases

Background:

  • Sickle hepatopathy involves liver dysfunction driven by intravascular sickling and sinusoidal occlusion.
  • Infections and autoimmunity are known triggers for sickle hepatopathy.
  • The association between Epstein-Barr virus (EBV) and sickle hepatopathy has not been previously reported.

Observation:

  • Two cases of acute sickle hepatopathy triggered by primary Epstein-Barr virus (EBV) infection in patients with hemoglobin SC (HbSC) disease are presented.
  • The first case involved a 14-year-old girl with HbSC disease experiencing hepatic sequestration crisis.
  • The second case involved a 16-year-old boy with HbSC disease who developed life-threatening intrahepatic cholestasis and multiorgan failure.

Findings:

  • Primary Epstein-Barr virus (EBV) infection can precipitate acute sickle hepatopathy in individuals with hemoglobin SC (HbSC) disease.
  • Hepatic sequestration crisis, responsive to simple erythrocyte transfusion, was observed in one patient.
  • Severe intrahepatic cholestasis with multiorgan failure occurred in the second patient, indicating a potentially life-threatening presentation.

Implications:

  • This study identifies a novel association between Epstein-Barr virus (EBV) infection and sickle hepatopathy, expanding the understanding of its triggers.
  • Recognizing EBV as a potential trigger is crucial for timely diagnosis and management of sickle hepatopathy, especially in patients with HbSC disease.
  • The diverse clinical spectrum of EBV-induced sickle hepatopathy underscores the need for vigilant monitoring and tailored treatment strategies.