SHM Converge Scientific Abstract ePoster Gallery-A Presing Diagnosis of an Unusual Encephalopathy in Hemophagocytic Lymphohistiocytosis

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This case describes a 37-year-old woman with hypertension and end-stage renal disease on hemodialysis who presented with fever, confusion, and weakness after recurrent Acinetobacter bacteremia. She was tachypneic, lethargic, febrile, and had a diffuse erythematous rash, prompting ICU transfer, intubation, and vasopressor support. Initial evaluation showed pancytopenia, elevated liver enzymes, hypertriglyceridemia, extremely high ferritin (>100,000), elevated LDH, and markedly increased soluble IL-2 receptor and CXCL9 levels. Imaging revealed hepatosplenomegaly and diffuse lymphadenopathy, while lumbar puncture and EEG were negative.<br /><br />Given the clinical picture, HLH was suspected. She underwent lymph node and bone marrow biopsies and was started on dexamethasone, later escalated to etoposide by hematology. Her persistent altered mental status led to neurologic evaluation; CT head and vEEG were unrevealing, but MRI showed restricted diffusion in the bilateral parietal lobes, left insular cortex, and left frontal region, raising concern for posterior reversible encephalopathy syndrome (PRES).<br /><br />The presentation highlights that HLH is a rare, life-threatening hyperinflammatory syndrome that can be triggered by infection and may mimic sepsis or diffuse inflammatory states. Although the relationship between HLH and PRES is not well established, this case suggests a possible association in adults. The key teaching point is to consider HLH early in patients with systemic inflammation, altered mental status, and pancytopenia, since prompt recognition and hematology consultation are essential.

Asset Subtitle

Eric C. Dulmovits

Meta Tag

Author List Ava-Dawn Gabbidon, Eric C. Dulmovits, Nevon Song

Category Clinical Vignettes

Concept Hemophagocytic Lymphohistiocytosis

Concept Fever

Concept Pancytopenia

Concept Hyperferritinemia

Concept Posterior Reversible Encephalopathy Syndrome

Distinguished Non-Finalist

Presenter Organization Northwell Health

Presenting Author Eric C. Dulmovits

Track Adult

Keywords

hemophagocytic lymphohistiocytosis

HLH

posterior reversible encephalopathy syndrome

PRES

Acinetobacter bacteremia

pancytopenia

hyperferritinemia

hepatosplenomegaly

altered mental status

dexamethasone etoposide

Hemophagocytic Lymphohistiocytosis

Fever

Pancytopenia

Hyperferritinemia

Posterior Reversible Encephalopathy Syndrome