The possibility of hemophagocytic lymphohistiocytosis should always be kept in mind when examining/treating a patient with fever of unknown origin and sepsis-like symptoms. Early diagnosis leading to prompt initiation of immunosuppressive therapy as well as aggressive supportive care, including correction of coagulation abnormalities and treatment of opportunistic infections, can decrease mortality.
Keywords: Abdominal compartment syndrome; Epstein–Barr virus; hemophagocytic lymphohistiocytosis; hypofibrinogenemia; invasive aspergillosis.