SHM Converge Scientific Abstract ePoster Gallery-A Rare Case of Idiopathic Hemophagocytic Lymphocytic Histiocytosis in Adults

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Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening syndrome characterized by multiorgan involvement and an excessive inflammatory response. It results from defective apoptosis and uncontrolled activation/proliferation of macrophages and lymphocytes, leading to a hyperimmune state. HLH can be primary or secondary; primary HLH is more common in children, while secondary HLH is more common in adults. In adults, secondary HLH is often associated with malignancies such as leukemia or lymphoma, infections including CMV, EBV, and HIV, and autoimmune or rheumatologic diseases such as SLE and RA.<br /><br />This report describes a rare case of idiopathic HLH in a 64-year-old previously healthy man who presented with fever, chills, generalized fatigue, and worsening jaundice. Laboratory evaluation revealed marked liver injury and cholestasis, including AST 458 U/L, ALT 227 U/L, bilirubin 8.3 mg/dL, and ALP 160 U/L. He was initially admitted with suspected sepsis and started on broad-spectrum antibiotics.<br /><br />The case highlights the diagnostic challenge of HLH in adults, particularly when the presentation mimics sepsis or other common inflammatory conditions. Despite being a severe syndrome with high mortality risk, this patient was managed conservatively and had a good outcome, emphasizing the importance of considering HLH in the differential diagnosis of unexplained systemic illness with liver dysfunction.

Asset Subtitle

Syed R. Shah

Meta Tag

Author List Alisa Seng, Benton Huang, Maninderjit Singh, Sobia Syed, Syed R. Shah

Category Clinical Vignettes

Concept Hemophagocytic Lymphohistiocytosis

Concept Secondary hemophagocytic lymphohistiocytosis

Concept Defective Apoptosis

Concept Hyperimmune Response

Concept Multiorgan Involvement

Distinguished Non-Finalist

Presenter Organization University of Washington

Presenting Author Syed R. Shah

Track Adult

Keywords

hemophagocytic lymphohistiocytosis

HLH

hyperinflammatory syndrome

multiorgan involvement

macrophage activation

adult HLH

idiopathic HLH

liver injury

cholestasis

sepsis mimic

Hemophagocytic Lymphohistiocytosis

Secondary hemophagocytic lymphohistiocytosis

Defective Apoptosis

Hyperimmune Response

Multiorgan Involvement