Introduction: Hemophagocytic lymph histiocytosis (HLH) is a rare, life-threatening hyperinflammatory syndrome triggered by infections, malignancies, or autoimmune diseases. It is typically associated with immunocompromised states but can also occur in immunocompetent individuals, making diagnosis challenging. We present a case of HLH triggered by cytomegalovirus (CMV) infection in a previously healthy adult, leading to multiorgan failure and severe acute respiratory distress syndrome (ARDS) Description: A 43-year-old previously healthy male nurse presented with a two-week history of cough and intermittent fevers. He was initially treated with levofloxacin for presumed community-acquired pneumonia, but symptoms worsened. On admission, he was febrile, tachypneic, and tachycardic. CT chest revealed bilateral pleural effusions and dependent atelectasis. He denied smoking, alcohol use, recent travel, or known exposures. Labs revealed leukopenia, thrombocytopenia, transaminitis, and positive CMV and EBV serologies. Despite broad-spectrum antibiotics, his condition deteriorated, requiring ICU transfer and mechanical ventilation. Karius testing confirmed CMV viremia; ganciclovir was initiated. He met HLH-2004 criteria: persistent fever, hepatosplenomegaly, pancytopenia, hyperferritinemia (ferritin >10,000 ng/mL), hypertriglyceridemia, and elevated soluble IL-2 receptor (sCD25). Ganciclovir-induced renal and hepatic toxicity required CRRT. His clinical course progressed to severe ARDS requiring proning. High-dose corticosteroids were started with gradual improvement. Over time, he was weaned off ventilatory and renal support and discharged with close hematology follow-up. Discussion: This case underscores the need to consider HLH in critically ill, immunocompetent adults presenting with persistent fevers, cytopenia’s, and multiorgan failure. CMV is a rare but important trigger of HLH in otherwise healthy hosts. Early recognition and timely initiation of immunosuppressive therapy are essential for improving outcomes in this potentially fatal condition.
Bansal et al. (Sun,) studied this question.