SHM Converge Scientific Abstract ePoster Gallery-Secondary Hlh: A Tale of an Eye Catching Ferritin

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Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hyperinflammatory syndrome caused by uncontrolled activation of histiocytes and T lymphocytes. It may be inherited or acquired, with triggers including infection, malignancy, and rheumatologic disease. Diagnosis is difficult because clinical criteria are nonspecific and often based on pediatric standards. Common clues include fever, cytopenias, hepatosplenomegaly, very high ferritin, elevated triglycerides, abnormal liver enzymes, and increased soluble IL-2 receptor levels. Without prompt recognition, HLH can progress to multi-organ failure.<br /><br />This case describes a 36-year-old man with no prior medical history who presented with 3 days of fever, nausea, vomiting, and diarrhea, along with hyponatremia and acute kidney injury. He was initially treated as having viral gastroenteritis, then later received ciprofloxacin and metronidazole when symptoms persisted. By hospital day 6, he developed elevated liver enzymes, pancytopenia, and ferritin of 4,450 ng/mL. His course worsened with multiple pulmonary emboli, heavy proteinuria, edema, and ascites. Infectious cultures were negative. Bone marrow biopsy showed hemophagocytosis, renal biopsy revealed focal segmental glomerulosclerosis (FSGS), and soluble IL-2 receptor was elevated.<br /><br />Given the high ferritin and evolving clinical picture, HLH was considered early. His HScore was 209, corresponding to an 88–93% probability of hemophagocytic syndrome. He was treated with prednisone 1 mg/kg and then transitioned to tocilizumab.<br /><br />The case emphasizes the importance of early suspicion for HLH in adults with unexplained fever, cytopenias, and marked hyperferritinemia. Early diagnosis is crucial because HLH causes severe systemic damage and carries significant mortality, even though secondary HLH has a better prognosis than primary HLH.

Asset Subtitle

Daniel W. Shields Jr.

Meta Tag

Author List Daniel W. Shields Jr., Joe an, Lidia Kruse-Peralta, Michael Dore

Category Clinical Vignettes

Concept Hemophagocytic Lymphohistiocytosis

Concept Fever

Concept Cytopenia

Concept H-score

Concept Hepatosplenomegaly

Distinguished Non-Finalist

Presenter Organization Naval Medical Center Portsmouth

Presenting Author Daniel W. Shields Jr.

Track Adult

Keywords

hemophagocytic lymphohistiocytosis

HLH

hyperinflammatory syndrome

hyperferritinemia

cytopenias

fever

hemophagocytosis

HScore

focal segmental glomerulosclerosis

soluble IL-2 receptor

Hemophagocytic Lymphohistiocytosis

Fever

Cytopenia

H-score

Hepatosplenomegaly