SHM Converge Scientific Abstract ePoster Gallery-Beyond Sepsis: A Case of Hemophagocytic Lymphohistiocytosis

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This case report describes a 31-year-old man with hemophagocytic lymphohistiocytosis (HLH), a rare but potentially fatal inflammatory syndrome caused by uncontrolled activation of macrophages and cytotoxic T-cells. HLH can closely resemble sepsis, which makes it difficult to recognize early, especially because there is no single test that confirms the diagnosis.<br /><br />The patient had a three-month history of recurrent fevers and had already undergone extensive outpatient evaluation without a clear diagnosis. In the emergency department, he was febrile, hypotensive, and tachycardic, so he was admitted for presumed sepsis of unknown cause. Laboratory studies showed anemia, low white blood cell count, thrombocytopenia, markedly elevated liver enzymes, high ferritin, low fibrinogen, and elevated soluble CD25. Imaging revealed splenomegaly. Additional testing showed a very high Epstein-Barr virus (EBV) DNA level, while blood cultures, HIV, CMV, hepatitis testing, ANA, ANCA, and RF were negative.<br /><br />These findings met HLH diagnostic criteria, supporting a diagnosis of EBV-triggered secondary HLH. The patient initially improved with intravenous immunoglobulin (IVIG) and dexamethasone, and then improved further after etoposide was added according to EBV-HLH treatment guidelines.<br /><br />The report emphasizes that HLH should be considered in patients who appear septic but have an unrevealing infectious workup, especially when fever, cytopenias, splenomegaly, hyperferritinemia, hypofibrinogenemia, and transaminitis are present. Early recognition of the underlying trigger and prompt, targeted treatment are critical to preventing organ failure and improving outcomes.

Asset Subtitle

Maria Louise P. Avenido

Meta Tag

Author List An Uche, Maria Louise P. Avenido, Vu D. Nguyen

Category Clinical Vignettes

Concept Hemophagocytic Lymphohistiocytosis

Concept HLH-04 criteria

Concept Cytopenias

Concept Hyperferritinemia

Concept Hyperinflammatory Syndrome

Distinguished Non-Finalist

Presenter Organization Highland Hospital

Presenting Author Maria Louise P. Avenido

Track Adult

Keywords

hemophagocytic lymphohistiocytosis

HLH

Epstein-Barr virus

EBV-triggered HLH

sepsis mimic

hyperferritinemia

cytopenias

splenomegaly

dexamethasone

etoposide

Hemophagocytic Lymphohistiocytosis

HLH-04 criteria

Cytopenias

Hyperferritinemia

Hyperinflammatory Syndrome