P2570 - Does the H Stand for Herpes Simplex or Hemophagocytic Lymphohistiocytosis? A Challenging Case of Acute Liver Failure

Safah Khan, ¹, Sarah Khan, MD², Malik Mushannen, MD³, Mohammad I. Bhatti, ¹, Preston Atteberry, MD⁴, David Wan, BS, MD^5
1Weill Cornell Medicine-Qatar, Doha, Ad Dawhah, Qatar; ²Cleveland Clinic Foundation, Cleveland, OH; ³NYP-Brooklyn Methodist Hospital, Brooklyn, NY; ⁴New York-Presbyterian Hospital/Weill Cornell Medical Center, New York, NY; ⁵Weill Cornell Medicine, New York, NY

Introduction: Herpes simplex virus (HSV) is a rare cause of acute liver failure (ALF), with a high mortality rate (90%) even in immunocompetent patients. Diagnosis can be difficult due to non-specific clinical presentation, hence a high index of clinical suspicion is warranted. Here we present a challenging case of HSV-1 induced ALF masquerading as hemophagocytosis lymphohistiocytosis (HLH).

Case Description/Methods: A 40-year old immunocompetent male was transferred to us with fevers, abdominal pain and sepsis which developed in 2 days. Labs were remarkable for AST >6000, ALT 1,856, WBC 1.1, platelets 14, INR 2.4, fibrinogen 80, ferritin >20,000, soluble IL2 receptor 4,624 and Cr 6.43 from baseline. Abdominal ultrasound noted only hepatosplenomegaly. The patient was started on IV methylprednisolone for presumed HLH. Due to the fevers, he was also started empirically on acyclovir. Within the first day, ferritin levels dropped to 365 though he continued to deteriorate clinically with worsening hemodynamic instability, respiratory failure, and encephalopathy despite addition of etoposide and emapalumab. On hospital day 4, bone marrow biopsy resulted without evidence of hemophagocytosis, and immunosuppressives were discontinued. Shortly thereafter, the HSV viral load resulted at 5.5 million, and HSV-1 DNA was detected from the mucous membrane lesions. Despite appropriate antiviral therapy, he continued to decline and eventually expired.

Discussion: HSV-induced ALF is rare (< 1%) and underrecognized, especially in the immunocompetent. Symptoms are vague with most patients lacking characteristic herpetic lesions; our patient had fevers and subtle orogenital lesions discovered only in retrospect. Diagnosis is also difficult due to overlap with other clinical entities. Our patient met 6/8 diagnostic criteria for HLH (Table 1) adding up to an H-score of 223 (96% predictive) and prompting early initiation of HLH therapy. However, his presentation could also be explained by disseminated HSV infection itself. Our case exemplifies a diagnostic conundrum as both viral infection and secondarily induced HLH present similarly with fevers, cytopenias, liver failure, DIC, and widespread cytokine abnormalities. Despite challenges, it is imperative to distinguish these two entities due to risks of worsening infection with HLH-directed immunosuppression. We recommend a high degree of suspicion for HSV-1 in febrile patients with ALF, even if immunocompetent, as early acyclovir therapy has the potential to improve outcomes.


Disclosures:


Safah Khan, ¹, Sarah Khan, MD², Malik Mushannen, MD³, Mohammad I. Bhatti, ¹, Preston Atteberry, MD⁴, David Wan, BS, MD⁵. P2570 - Does the H Stand for Herpes Simplex or Hemophagocytic Lymphohistiocytosis? A Challenging Case of Acute Liver Failure, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.