Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hematological disorder characterized by immune dysregulation with multiple organ involvement and carries a poor prognosis. The occurrence of HLH can be familial or sporadic, which is triggered by causes like infection or malignancy. This case report is about a 47-year-old male who presented to the hospital with a fever, chills, night sweats, and unintentional weight loss. He was found to have severely elevated ferritin, and computed tomography showed cirrhosis, a normal sized spleen, and retroperitoneal lymphadenopathy. He underwent an extensive battery of tests to identify the etiology. Meanwhile, he had recurrent fevers with worsening transaminitis and septic shock, requiring admission to the ICU. Blood tests for Epstein-Barr virus (EBV) deoxyribonucleic acid (DNA) and immunoglobulin G (IgG) were positive. Due to high suspicion of HLH, he was started on intravenous methylprednisone 1000 mg daily for three days with clinical improvement. A bone marrow biopsy showed hemophagocytosis and he was diagnosed with EBV-associated HLH. He was continued on steroids with oral prednisone and continued to clinically improve. He was later tapered off steroids over the course of five months. HLH is a rapidly progressive and fatal condition that requires prompt treatment, and thus a high index of suspicion is needed to make a timely diagnosis.