1 Department of Internal Medicine, Hayatabad Medical Complex (HMC), MTI, Peshawar, Khyber Pakhtunkhwa, Pakistan.
2 Department of Internal Medicine, Khyber Teaching Hospital (KTH), MTI, Shangla, Khyber Pakhtunkhwa, Pakistan.
3 Department of Internal Medicine, Ayub Teaching Hospital (ATH), MTI, Abbottabad, Khyber Pakhtunkhwa, Pakistan.
4 Department of Internal Medicine, Northwest Health Porter, Valparaiso, Indiana, USA.
5 Department of Internal Medicine, Jewish Hospital Cincinnati, Cincinnati, Ohio, USA.
6 Department of Internal Medicine, Hayatabad Medical Complex (HMC), MTI, Peshawar, Khyber Pakhtunkhwa, Pakistan.
World Journal of Advanced Research and Reviews, 2025, 27(03), 1228-1230
Article DOI: 10.30574/wjarr.2025.27.3.3187
Received on 11 August 2025; revised on 14 September 2025; accepted on 18 September 2025
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.
Hemophagocytic Lymphohistiocytosis (HLH); Secondary HLH; Hepatosplenomegaly; Hyperferritinemia; HLH-2004 diagnostic criteria; HScore; HLH-2004 regimen; Immunosuppressive therapy; Adult HLH
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Dawood Khan, Muhammad Aamir Iqbal, Hasnat Ahmed, Abdul Mueed Bangash, Muhammad Saad and Muhammad Talha. Secondary Hemophagocytic Lymphohistiocytosis (HLH) in a 40-Year-Old Female: A rare clinical presentation highlighting the importance of early recognition and management.World Journal of Advanced Research and Reviews, 2025, 27(03), 1228-1230. Article DOI: https://doi.org/10.30574/wjarr.2025.27.3.3187.
Copyright © 2025 Author(s) retain the copyright of this article. This article is published under the terms of the Creative Commons Attribution Liscense 4.0