Infection-associated hemophagocytic lymphohistiocytosis: a case report

Abstract

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory disorder characterized by fever, cytopenia, splenomegaly, and hemophagocytosis. Without prompt treatment, HLH can rapidly progress to life-threatening multiorgan failure. The authors present a case of occult HLH with severe bicytopenia and organ dysfunction requiring intensive care.

Case presentation: A 20-year-old male presented with fever, cough, and constitutional symptoms. He developed hypoxia, elevated transaminases, and bicytopenia. Despite transfusions, platelet counts remained critically low. With high suspicion for HLH, head computed tomography and bone marrow biopsy was although panned but couldn't be performed due to resource less settings. And with suspicion for HLH treatment with high-dose dexamethasone was initiated as counts improved.

Clinical course: The patient required mechanical ventilation for pulmonary infiltrates. He exhibited seizure activity and epistaxis related to coagulopathy. On hospital day 9, he was successfully extubated as counts normalized. He was discharged from the intensive care unit once stable.

Conclusion: This case illustrates a delayed diagnosis of HLH masquerading as a fever of unknown origin. HLH should be urgently considered in patients with unexplained cytopenia, organ dysfunction, and systemic inflammation. Early treatment with immunotherapy can be lifesaving, whereas delays may precipitate irreversible end-organ damage.

Keywords: HLH; case report; hemophagocytic lymphohistiocytosis; immunotherapy; organ failure; pancytopenia.