Sickle hepatopathy comprises a spectrum of disorders that vary in severity. Intravascular sickling and sinusoidal occlusion are the principal drivers of sickle hepatopathy, but infection or autoimmunity can act as triggers. We describe two cases of acute sickle hepatopathy initiated by primary Epstein–Barr virus (EBV) infection, a previously unreported association. The first case entailed a 14-year-old girl with hemoglobin SC (HbSC) disease who developed hepatic sequestration crisis that responded to a simple transfusion of erythrocytes. The second case was that of a 16-year-old boy with HbSC disease who experienced life-threatening intrahepatic cholestasis with multiorgan failure.

Original languageEnglish
Article numbere29196
JournalPediatric Blood and Cancer
Issue number9
StatePublished - Sep 2021


  • cholestasis
  • hemoglobin SC disease
  • hepatic sequestration
  • infectious mononucleosis
  • sickle cell disease


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