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Hemophagocytic lymphohistiocytosis (HLH) is a cytokine storm syndrome caused by an overactive but ineffective immune reaction. Without prompt diagnosis and treatment, HLH is life-threatening. However, presenting symptoms are often nonspecific, with fatigue and fever being the most common. A high index of suspicion is therefore critical for early diagnosis and timely management. A previously healthy, 65-year-old female who initially presented with fever and abdominal pain developed abdominal compartment syndrome (ACS) requiring decompressive laparotomy on hospital day 6. Intraoperative frozen sections of biopsied liver showed intense portal lymphohistiocytic infiltrates. Epstein-Barr virus DNA copy numbers escalated from 600 copies/ mL after admission to 134,000 copies/mL before death. The diagnostic criteria of HLH-2004 were met. Patient expired on hospital day 12. It is important to raise awareness of ACS being an unusual presentation of HLH. Recent changes in diagnostic criteria tailored to adult HLH cases are reviewed.

Epstein-Barr virus associated secondary hemophagocytic lymphohistiocytosis with an unusual presentation of abdominal compartment syndrome