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

Year: 2025

Type: article

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.

Source: Zenodo (CERN European Organization for Nuclear Research)

DOI: 10.5281/zenodo.17761053

Authors: Khan, Dawood, Iqbal, Muhammad Aamir, Ahmed, Hasnat, Bangash, Abdul Mueed, Saad, Muhammad, Talha, Muhammad

Institutions: Hayatabad Medical Complex, Khyber Teaching Hospital, Community Health Systems - Northwest Health - Porter, Jewish Hospital

Language: English

Topic: Autoimmune and Inflammatory Disorders Research

Subfield: Hematology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: green

OpenAlex ID: W7107960824

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