HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS PRESENTING AS PERSISTENT FEVER AND PANCYTOPENIA: A RARE CASE REPORT

Abstract

Background: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder of excessive activation of macrophages and lymphocytes that can result in overwhelming inflammatory responses, multi-organ failure, and death. The nonspecific symptoms and signs of HLH can lead to significant diagnostic delays Case Presentation: A 42-year-old male was admitted with high-grade fevers, fatigue, anorexia, weight loss and progressing pancytopenia. Physical examination was notable for pallor and hepatosplenomegaly. Work-up was positive for severely reduced blood counts, markedly elevated ferritins (18,500 ng/mL), triglycerides, and decreased fibrinogen; liver enzymes and lactate dehydrogenase were elevated. Work-up for infection, autoimmunity, and malignancy was negative. Bone marrow biopsy demonstrated considerable hemophagocytosis. This patient met the diagnostic criteria for HLH-2004 and had a diagnosis of secondary HLH. The patient responded to dexamethasone and etoposide treatment in combination with general supportive care. Conclusion: HLH should be considered in patients presenting with prolonged fever, cytopenias, and unexplained systemic inflammation. Early recognition, prompt diagnostic evaluation, and timely initiation of therapy are essential for improving survival and preventing disease-related complications.