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Background <p>Hemophagocytic Lymphohistiocytosis (HLH) is a syndrome characterized by severe hyperinflammation and immune dysfunction with concomitant immune system activation. HLH often requires supportive care management in Intensive Care Unit (ICU) with high mortality rates (36–40% of pediatric cases and 41–68% of adult cases). HLH diagnosis in ICU may be challenging due to lack of specific laboratory, radiologic and histopathologic findings, and above all the overlap of its clinical features with Multi-Organ Dysfunction syndrome (MOD). Here we present clinical characteristics, possible triggers and outcomes of a cohort of six patients admitted to our ICU for HLH or HLH-induced MOD.</p> Methods <p>Here we present clinical characteristics, possible triggers and outcomes retrospectively collected of a cohort of patients admitted to our ICU for HLH or HLH-induced MOD. The study has been approved by the local ethics committee as part of the PROSAFE data collection project.</p> Results <p>Six patients were included. All but one patient had a HScore with high probability of HLH, even when not all HScore items were tested. We found infection as a potential trigger in all patients. All patients died before hospital discharge.</p> Conclusion <p>HLH is a condition that can overlap clinical features of septic shock. Although rare it should be considered in differential diagnosis of distributive shock without established etiology.</p>

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Intensive Care management of Hemophagocytic Lymphohistiocytosis: a case series

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年11月14日

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  • Riccardo Traversi,
  • Gabriele Arrigo,
  • Chiara Bonetto,
  • Gabriele Sales,
  • Alessandro Busca,
  • Vito Fanelli,
  • Claudia Filippini,
  • Luca Brazzi,
  • Giorgia Montrucchio

摘要

Background

Hemophagocytic Lymphohistiocytosis (HLH) is a syndrome characterized by severe hyperinflammation and immune dysfunction with concomitant immune system activation. HLH often requires supportive care management in Intensive Care Unit (ICU) with high mortality rates (36–40% of pediatric cases and 41–68% of adult cases). HLH diagnosis in ICU may be challenging due to lack of specific laboratory, radiologic and histopathologic findings, and above all the overlap of its clinical features with Multi-Organ Dysfunction syndrome (MOD). Here we present clinical characteristics, possible triggers and outcomes of a cohort of six patients admitted to our ICU for HLH or HLH-induced MOD.

Methods

Here we present clinical characteristics, possible triggers and outcomes retrospectively collected of a cohort of patients admitted to our ICU for HLH or HLH-induced MOD. The study has been approved by the local ethics committee as part of the PROSAFE data collection project.

Results

Six patients were included. All but one patient had a HScore with high probability of HLH, even when not all HScore items were tested. We found infection as a potential trigger in all patients. All patients died before hospital discharge.

Conclusion

HLH is a condition that can overlap clinical features of septic shock. Although rare it should be considered in differential diagnosis of distributive shock without established etiology.