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.

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Publication Details

Journal
Zenodo (CERN European Organization for Nuclear Research)
Published
2025-09-30
DOI
https://doi.org/10.5281/zenodo.17761054
Primary Topic
Autoimmune and Inflammatory Disorders Research
Type
article
Field-Weighted Citation Impact
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article

Secondary Hemophagocytic Lymphohistiocytosis (HLH) in a 40-Year-Old Female: A rare clinical presentation highlighting the importance of early recognition and management

Zenodo (CERN European Organization for Nuclear Research)
Autoimmune and Inflammatory Disorders Research
article

Secondary Hemophagocytic Lymphohistiocytosis (HLH) in a 40-Year-Old Female: A rare clinical presentation highlighting the importance of early recognition and management

article en

Abstract

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.

Zenodo (CERN European Organization for Nuclear Research)
Ayub Medical College (PK), Valparaiso University (US), Khyber Teaching Hospital (PK), Hayatabad Medical Complex (PK), Jewish Hospital (US)
Gender equality, Good health and well-being
Openalex Percentile: Top 30%
Autoimmune and Inflammatory Disorders Research
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