A Rare and Fatal Complication of Herpes Simplex Virus (HSV) Infection
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Publication Date
4-29-2026
Keywords
oregon, psvmc, psvmc gme, psvmc oaa
Disciplines
Medical Education
Abstract
Introduction: Hemophagocytic Lymphohistiocytosis (HLH) is a rare syndrome characterized by overactive immune activation, cytokine storm, and uncontrolled hemophagocytosis. Primary HLH is familial and most often seen in infants, whereas secondary HLH occurs more frequently in adults and is triggered by infections, malignancies, or autoimmune disorders. Clinically, secondary HLH often mimics sepsis but fails to respond to standard therapies, contributing to delayed diagnosis and treatment. Without timely intervention, HLH can result in extensive tissue damage, multiorgan failure, and death. In rare cases, central nervous system (CNS) involvement may occur, manifesting as histiocytic infiltration that can be misinterpreted as malignancy or infection. Case Presentation: A 51 year-old man presented with four days of nausea, vomiting, diarrhea, and fevers. Initial evaluation revealed persistent hypotension, severe transaminitis, neutropenia, thrombocytopenia, and penile lesions, prompting ICU admission. Hemodynamic stability was achieved with fluid administration and empiric antibiotics, after which the patient was transferred to the general ward and started on acyclovir following positive HSV results from genital lesion swabs and CSF samples. Subsequently, the patient developed acute renal failure, encephalopathy, and hepatic failure, suspected to be secondary to HSV hepatitis, necessitating ICU readmission. Due to the severity of organ dysfunction, N-acetyl-cysteine and continuous renal replacement therapy (CRRT) were initiated. Although HLH was initially considered unlikely due to its rare association with HSV, further evaluation confirmed the diagnosis. The patient was treated with high-dose steroids and etoposide chemotherapy, resulting in clinical improvement and transfer out of critical care. However, the patient later experienced recurrent encephalopathy. Brain MRI revealed multifocal lesions suggestive of HLH related infiltrative disease. Neurologic deterioration progressed to anisocoria and loss of brainstem reflexes, and repeat CT imaging revealed new lesions with mass effect and brain stem compression. Despite aggressive management, the patient succumbed to the disease. Discussion: This case underscores a complex diagnostic and therapeutic challenge involving a rare etiology of HLH. The clinical overlap between HLH and sepsis, both capable of causing multiorgan failure, contributes to under-recognition and delayed treatment. Initial management focused on treating HSV infection and its complications, which inadvertently postponed HLH diagnosis. Delayed recognition and initiation of immunosuppressive therapy, including corticosteroids and etoposide, are associated with increased mortality. Conclusions: This case highlights the importance of maintaining a high index of suspicion for HLH in patients with severe systemic illness, particularly when clinical deterioration persists despite appropriate treatment for presumed sepsis. Early identification and prompt initiation of HLH-directed therapy are critical to improving outcomes.
Specialty/Research Institute
Graduate Medical Education
Specialty/Research Institute
Internal Medicine
