Clinical-Pathological Conference Series from the Medical University of Graz

Keywords Hemophagocytic lymphohistiocytosis • Bicytopenia • Hyperferritinemia • Hyperinflammation • Hepatosplenomegaly Presentation of caseDr. S. Hatzl: The previously healthy plumber, who is married and has two healthy children (a boy and a girl), presented at a regional hospital 5 days prior to admission to the University Medical Center Graz with jaundice following episodes of fever (up to 40 °C) and sore throat.Laboratory results at the referring hospital: total bilirubin 21.9 mg/dL (normal: < 1.25 mg/dL), gamma-glutamyl transferase (GGT) 1424 U/L (normal: < 55 U/L), aspartate aminotransferase (AST) 740 U/L (normal: < 50 U/L), alanine aminotransaminase (ALT) 922 U/L (normal: < 60 U/L), and lactate dehydrogenase (LDH) 6230 U/L (normal: 120-240 U/L).There was no intrahepatic or extrahepatic bile duct dilatation on abdominal ultrasound and endoscopic retrograde cholangiopancreatography (ERCP) showed normal anatomical findings; however, a transpapillary stent was placed due to marked hyperbilirubinemia.Serological testing revealed cytomegalovirus (CMV) immunoglobulin (Ig)G and IgM positivity, and treatment with ganciclovir was initiated.Subsequently, the patient developed bicytopenia and coagulopathy, with