SHM Converge Scientific Abstract ePoster Gallery-Hospitalists Nightmare

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This case series describes three adult patients who initially appeared to have severe sepsis with multiorgan failure but were ultimately diagnosed with hemophagocytic lymphohistiocytosis (HLH), a rare and often fatal hyperinflammatory syndrome.<br /><br />Case 1 involved a 40-year-old man with anemia, thrombocytopenia, renal failure, jaundice, elevated liver enzymes, hepatosplenomegaly, coagulopathy, and very high ferritin and LDH. Infectious testing later showed elevated EBV DNA.<br /><br />Case 2 involved a 69-year-old woman with a history of ANCA vasculitis who presented with fever, chills, arthralgias, headache, and cough. She was treated for suspected sepsis, but cultures and viral testing were negative. Her condition worsened with respiratory failure and bilateral infiltrates, leading to intubation. She had elevated liver enzymes, thrombocytopenia, hyponatremia, acute kidney injury, hematuria, proteinuria, ferritin over 54,000 ng/mL, hypertriglyceridemia, and hypofibrinogenemia. HLH was suspected rather than vasculitis relapse.<br /><br />Case 3 involved a 72-year-old woman with abdominal pain, acute renal failure, lactic acidosis, thrombocytopenia, markedly abnormal liver tests, DIC, pancytopenia, ferritin of 31,000 ng/mL, and high LDH. Infectious workup identified Ehrlichia DNA.<br /><br />Bone marrow biopsy in all three cases showed hemophagocytosis, and soluble IL-2 receptor levels were elevated in cases 2 and 3. Despite treatment with etoposide and dexamethasone in some patients, two of the three died.<br /><br />The authors emphasize that adult HLH is frequently mistaken for sepsis because it presents with nonspecific findings such as fever, cytopenias, organ dysfunction, and inflammatory markers. Early recognition is crucial because mortality is high and delayed diagnosis can be fatal.

Asset Subtitle

Atchuyta Pachigolla

Meta Tag

Author List Atchuyta Pachigolla, Chris Snyder, Srujana Pachigolla, Suresh Kumar Ponnada

Category Clinical Vignettes

Concept Hemophagocytic Lymphohistiocytosis

Concept Cytopenia

Concept Hyperferritinemia

Concept Sepsis

Concept Fever

Distinguished Non-Finalist

Presenter Organization University of Nebraska Medical Center

Presenting Author Atchuyta Pachigolla

Track Adult

Keywords

hemophagocytic lymphohistiocytosis

HLH

sepsis mimic

multiorgan failure

ferritin

hemophagocytosis

EBV infection

Ehrlichia infection

cytopenias

adult hyperinflammatory syndrome

Hemophagocytic Lymphohistiocytosis

Cytopenia

Hyperferritinemia

Sepsis

Fever