Fatal hemophagocytic syndrome associated with hepatitis B virus infection: Case report and review of the literature

A 16-year old boy was admitted with a 20 day history of fever, loss of appetite, jaundice and dark urine, and recent epistaxis. Physical examination revealed icteric sclera, generalized lymphadenopathy, diffuse abdominal tenderness and marked hepatosplenomegaly. Laboratory findings showed bicytopenia, coagulopathy, hypofibrinogenemia and hypoproteinemia in addition to elevated ESR, C-reactive protein, serum bilirubin, transaminases, alkaline phosphates, lactic dehydrogenase, gamma-glutamyl transpeptidase, and triglyceride levels. Hepatitis-B surface antigen and hepatitis B core IgM antibody were positive, while lymph node biopsy demonstrated infiltration with histiocytic series displaying marked erythrophagocytosis. The patient was diagnosed with hepatitis B virus infection-associated hemophagocytic syndrome. The condition deteriorated rapidly and he died 12 days after admission. This report suggests that in patients with hepatitis B infection presenting unexplained fever, localized bleeding due to thrombocytopenia and showing a rapid clinical deterioration, early bone marrow or lymph node biopsy for diagnosis and intensive therapy should be performed.