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Background <p>Enteric fever remains a major public health concern in South Asia, accounting for more than two-thirds of the global burden. Although most cases follow a self-limiting course with timely antimicrobial therapy, a minority develop severe complications. Hemophagocytic lymphohistiocytosis (HLH) and acute respiratory distress syndrome (ARDS) are rare but potentially fatal manifestations, and their co-occurrence in typhoid fever is exceptionally uncommon.</p> Case Presentation <p>We report the case of adolescent with culture-proven Salmonella enterica serovar Typhi infection who presented with persistent fever and rapidly progressive hypoxemia despite appropriate antimicrobial therapy. Laboratory evaluation revealed bicytopenia, hyperferritinemia, hypofibrinogenemia, and elevated liver enzymes. The HScore was 254, corresponding to a &gt; 99% probability of HLH. Bone marrow aspirate confirmed hemophagocytosis. The patient was diagnosed with secondary HLH and moderate ARDS and managed with culture-directed antibiotics and immunosuppressive therapy (dexamethasone), resulting in clinical improvement.</p> Discussion <p>HLH should be considered in patients with enteric fever who deteriorate despite appropriate antimicrobial therapy, particularly when cytopenia and hyperinflammatory markers are present. The use of standardized diagnostic criteria such as HLH-2004 and HScore facilitates timely recognition.</p> Conclusion <p>This case highlights the importance of early recognition of HLH as a rare but serious complication of typhoid fever. Prompt initiation of targeted therapy can be lifesaving, especially when complicated by respiratory failure.</p>

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The Cytokine Crossfire - Secondary Hemophagocytic Lymphohistiocytosis and Ards Complicating Enteric Fever: A Case Report

  • 期刊论文

  • 发表日期：

    2026年2月18日

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  • Vignesh Jayaprakash,
  • Shibu Sasidharan,
  • Yasmin Muhammad,
  • Aayushi Sharma

摘要

Background

Enteric fever remains a major public health concern in South Asia, accounting for more than two-thirds of the global burden. Although most cases follow a self-limiting course with timely antimicrobial therapy, a minority develop severe complications. Hemophagocytic lymphohistiocytosis (HLH) and acute respiratory distress syndrome (ARDS) are rare but potentially fatal manifestations, and their co-occurrence in typhoid fever is exceptionally uncommon.

Case Presentation

We report the case of adolescent with culture-proven Salmonella enterica serovar Typhi infection who presented with persistent fever and rapidly progressive hypoxemia despite appropriate antimicrobial therapy. Laboratory evaluation revealed bicytopenia, hyperferritinemia, hypofibrinogenemia, and elevated liver enzymes. The HScore was 254, corresponding to a > 99% probability of HLH. Bone marrow aspirate confirmed hemophagocytosis. The patient was diagnosed with secondary HLH and moderate ARDS and managed with culture-directed antibiotics and immunosuppressive therapy (dexamethasone), resulting in clinical improvement.

Discussion

HLH should be considered in patients with enteric fever who deteriorate despite appropriate antimicrobial therapy, particularly when cytopenia and hyperinflammatory markers are present. The use of standardized diagnostic criteria such as HLH-2004 and HScore facilitates timely recognition.

Conclusion

This case highlights the importance of early recognition of HLH as a rare but serious complication of typhoid fever. Prompt initiation of targeted therapy can be lifesaving, especially when complicated by respiratory failure.