Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a clinical syndrome resulting from the immunological hyperactivation of macrophages. The condition can be triggered by an infection, malignancy, or autoimmune disease. Though the current literatures demonstrated a significant portion of sepsis patients are probably suffering from HLH.
Case description: Here, we present a case who presented like features of gram-negative sepsis but prompt diagnosis of HLH and treatment saved the patient\'s life.
Conclusion: Hemophagocytic lymphohistiocytosis should be anticipated and recognized in fulminating sepsis-like conditions and promptly treated with steroids.
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