SHM Converge Scientific Abstract ePoster Gallery-Hemophagocytic Lymphohistiocytosis Secondary to Epstein-Barr Virus: A Common Cause of a Rare Disease

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This case describes a 59-year-old woman with fatigue, weight loss, pancytopenia, and concern for hematologic malignancy who was ultimately diagnosed with hemophagocytic lymphohistiocytosis (HLH) secondary to Epstein-Barr virus (EBV) infection.<br /><br />HLH is a rare, life-threatening syndrome caused by overactivation of cytotoxic T cells, natural killer cells, and macrophages, leading to systemic inflammation, cytopenias, and organ injury. It may be primary, due to genetic defects, or secondary to triggers such as infections, malignancy, or autoimmune disease. Diagnosis is clinical and requires five of eight criteria, which include fever, splenomegaly, cytopenias, high ferritin, elevated soluble CD25, low NK-cell activity, hemophagocytosis, hypertriglyceridemia, and hypofibrinogenemia.<br /><br />The patient’s workup showed leukopenia, anemia, thrombocytopenia, very high ferritin, low fibrinogen, markedly elevated LDH, and high EBV viral load. Bone marrow biopsy showed no increased blasts, helping exclude leukemia. Cytokine testing revealed elevated inflammatory markers, and she also had positive autoimmune serologies, adding to the broad differential.<br /><br />She was treated with IV corticosteroids, ganciclovir, and intrathecal methotrexate because of central nervous system involvement. By discharge, her EBV PCR became undetectable and her blood counts stabilized, with close outpatient hematology follow-up arranged.<br /><br />The key takeaway is that HLH can present with nonspecific symptoms and mimic infection, autoimmune disease, or cancer. Although rare, it must be considered in EBV-associated illness because early recognition and aggressive treatment are essential to prevent poor outcomes.

Asset Subtitle

Cassandra L. Pasadyn

Meta Tag

Author List Cassandra L. Pasadyn, Kaitlyn Greiner

Category Clinical Vignettes

Concept Secondary hemophagocytic lymphohistiocytosis

Concept Cytopenia

Concept Fever

Concept Hyperferritinemia

Concept Cytotoxic T Cells

Distinguished Non-Finalist

Presenter Organization The Ohio State University College of Medicine

Presenting Author Cassandra L. Pasadyn

Track Adult

Keywords

hemophagocytic lymphohistiocytosis

HLH

Epstein-Barr virus

EBV infection

pancytopenia

ferritin

bone marrow biopsy

cytopenias

corticosteroids

intrathecal methotrexate

Secondary hemophagocytic lymphohistiocytosis

Cytopenia

Fever

Hyperferritinemia

Cytotoxic T Cells