SHM Converge Scientific Abstract ePoster Gallery-Immunosuppression Is Not Always the Answer

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This poster describes a rare case of Epstein–Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (HLH) in an otherwise healthy 27-year-old man. EBV-triggered HLH is usually seen in patients with immunosuppression or hematologic malignancy, so its occurrence in immunocompetent individuals is uncommon. Because HLH is a rapidly progressive inflammatory syndrome with high mortality, early recognition is critical.<br /><br />The patient presented with five days of fever, night sweats, sore throat, headache, vomiting, and neck pain. On exam he was febrile, tachycardic, and had mild splenomegaly. Initial differential diagnoses included meningitis, viral infection, autoimmune disease, hematologic malignancy, and DIC. Extensive infectious and autoimmune testing was unrevealing except for a positive monospot test.<br /><br />As his condition worsened, clinicians suspected EBV-associated HLH and started empiric ganciclovir (5 mg/kg IV every 12 hours). Further testing showed active EBV infection with EBV DNA PCR of 51,102 copies/mL. Bone marrow biopsy demonstrated hemophagocytosis, and soluble IL-2 receptor levels were markedly elevated (35,125 pg/mL), supporting the diagnosis of HLH. His H score was 228.<br /><br />The case highlights that EBV-induced HLH should be considered in patients with persistent fever, cytopenias, abnormal liver function tests, and very high ferritin or triglycerides. Treatment should target the underlying trigger; in this case, antiviral therapy was associated with successful recovery. The authors emphasize that more research is needed to define the role of antivirals in infection-triggered HLH and to better understand outcomes in clinically stable patients.

Asset Subtitle

Kirtan N. Patolia

Meta Tag

Author List Kirtan N. Patolia, Mihir Shah, Sandhya-Shri Kannayiram

Category Clinical Vignettes

Concept Hemophagocytic Lymphohistiocytosis

Concept Epstein-Barr Virus

Concept Persistent Fever

Concept Cytopenia

Concept Ferritin

Distinguished Non-Finalist

Presenter Organization John H. Stroger, Jr. Hospital of Cook County

Presenting Author Kirtan N. Patolia

Track Adult

Keywords

Epstein-Barr virus

EBV-associated HLH

hemophagocytic lymphohistiocytosis

immunocompetent

fever

splenomegaly

bone marrow biopsy

hemophagocytosis

ganciclovir

soluble IL-2 receptor

Hemophagocytic Lymphohistiocytosis

Epstein-Barr Virus

Persistent Fever

Cytopenia

Ferritin