HLH IN DISGUISE: A RARE BUT FATAL HYPERINFLAMMATORY SYNDROME IN AN ADULT MALE

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is an uncommon and rapidly progressive hyperinflammatory syndrome that frequently mimics sepsis or hematologic malignancy, leading to delays in diagnosis. We report a 63-year-old male with bullous pemphigoid on chronic corticosteroid therapy and long-standing type 2 diabetes mellitus who presented with persistent high-grade fever, progressive cytopenias, altered sensorium, and hepatosplenomegaly. Further investigations revealed marked hyperferritinaemia, hypertriglyceridaemia, transaminitis, and hypofibrinogenaemia. Infectious and autoimmune causes were excluded after extensive evaluation. Bone marrow examination demonstrated hemophagocytosis with granulomatous inflammation, confirming HLH. The patient was managed with high-dose corticosteroids, intravenous immunoglobulin (IVIG), broad-spectrum antibiotics, and intensive supportive care. Over the following weeks, his inflammatory markers progressively improved, sensorium normalized, and cytopenias began to recover. He was discharged in a stable condition with scheduled outpatient follow-up. This case emphasizes the diagnostic complexity of adult-onset HLH in immunocompromised individuals and highlights the value of early recognition and timely immunomodulatory therapy in improving clinical outcomes.