Secondary Hemophagocytic Lymphohistiocytosis (HLH) in a 40-Year-Old Female: A rare clinical presentation highlighting the importance of early recognition and management
Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially life-threatening hyperinflammatory syndrome characterized by dysregulated immune activation. We report a case of secondary HLH in a 40-year-old woman with persistent high fever, pancytopenia, hepatosplenomegaly, and hyperferritinemia. The diagnosis was confirmed according to the HLH-2004 diagnostic criteria combined with HScore of 303 (corresponding probability > 99%). The patient responded well to the HLH-2004 regimen of immunosuppressive therapy (dexamethasone, etoposide, and cyclosporine A). This case suggests the importance of maintaining high clinical vigilance and timely intervention in the diagnosis and treatment of adult HLH.
Institutions
- Ayub Medical College (PK)
- Valparaiso University (US)
- Khyber Teaching Hospital (PK)
- Hayatabad Medical Complex (PK)
- Jewish Hospital (US)
Publication Details
- Journal
- Zenodo (CERN European Organization for Nuclear Research)
- Published
- 2025-09-30
- DOI
- https://doi.org/10.5281/zenodo.17761053
- Primary Topic
- Autoimmune and Inflammatory Disorders Research
- Type
- article
- Field-Weighted Citation Impact
- 0.00