SHM Converge Scientific Abstract ePoster Gallery-Rare Initial Presentation With Acute Liver Injury in Hemophagocytic Lymphohistiocytosis

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This case describes a rare presentation of hemophagocytic lymphohistiocytosis (HLH) with acute liver injury in an 18-year-old woman with no significant prior medical history. She presented with two weeks of abdominal pain, jaundice, nausea, vomiting, and fever after recent exudative tonsillitis and acetaminophen use. On admission, she had fever, tachycardia, jaundice, scleral icterus, and right upper quadrant tenderness. Laboratory studies showed pancytopenia and worsening liver tests, while CT imaging demonstrated hepatosplenomegaly.<br /><br />Because of persistent fever, splenomegaly, cytopenias, hyperferritinemia, elevated triglycerides, low fibrinogen, high LDH, and markedly elevated soluble IL-2 receptor levels, HLH was strongly suspected. Infectious and autoimmune workup identified Epstein-Barr virus (EBV) infection. Bone marrow and liver biopsies confirmed hemophagocytosis, and EBER-ISH staining was positive in both tissues. A clonal cytotoxic T-cell population was also found, supporting the diagnosis of HLH.<br /><br />The patient was treated with methylprednisone and rituximab, leading to clinical improvement and a gradual decrease in liver enzymes and bilirubin. She was discharged on a prednisone taper and later had complete symptom resolution with normalization of laboratory values.<br /><br />The report highlights that HLH is a rare, life-threatening immune dysregulation syndrome that is difficult to diagnose. Although liver involvement is common, presentation with severe acute liver injury is unusual. The case emphasizes the importance of recognizing HLH early, especially in EBV-associated disease, to guide timely treatment and improve outcomes.

Asset Subtitle

Zin T. Win

Meta Tag

Author List Khaled Al Smadi, Mason Matsubara, Umair Khizer, Zin T. Win

Category Clinical Vignettes

Concept Hemophagocytic Lymphohistiocytosis

Concept Immune Dysregulation

Concept Cytokine Release Syndrome

Concept Epstein-Barr Virus

Concept Cytopenia

Distinguished Non-Finalist

Presenter Organization University of California Riverside

Presenting Author Zin T. Win

Track Adult

Keywords

hemophagocytic lymphohistiocytosis

HLH

acute liver injury

Epstein-Barr virus

EBV infection

hepatosplenomegaly

pancytopenia

hyperferritinemia

hemophagocytosis

rituximab

Hemophagocytic Lymphohistiocytosis

Immune Dysregulation

Cytokine Release Syndrome

Epstein-Barr Virus

Cytopenia