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Haemophagocytic lymphohistiocytosis in adult critical care

Authors
  • Bauchmuller, Kris1
  • Manson, Jessica J2
  • Tattersall, Rachel3, 4
  • Brown, Michael5, 6
  • McNamara, Christopher7
  • Singer, Mervyn8
  • Brett, Stephen J9
  • 1 Department of Critical Care and Anaesthesia, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK
  • 2 Department of Rheumatology, University College London Hospitals NHS Foundation Trust, London, UK
  • 3 Department of Rheumatology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK
  • 4 Department of Paediatric and Adolescent Rheumatology, Sheffield Children’s NHS Trust, Sheffield, UK
  • 5 Hospital for Tropical Diseases, University College London Hospitals NHS Foundation Trust, London, UK
  • 6 Clinical Research Department, London School of Hygiene and Tropical Medicine, London, UK
  • 7 Department of Haematology, University College London Hospitals NHS Foundation Trust, London, UK
  • 8 Division of Medicine, Bloomsbury Institute of Intensive Care Medicine, University College London, London, UK
  • 9 Department of Surgery and Cancer, Imperial College London, London, UK
Type
Published Article
Journal
Journal of the Intensive Care Society
Publisher
SAGE Publications
Publication Date
Jan 07, 2020
Volume
21
Issue
3
Pages
256–268
Identifiers
DOI: 10.1177/1751143719893865
PMID: 32782466
PMCID: PMC7401439
Source
PubMed Central
Keywords
License
Unknown

Abstract

Haemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe immune dysregulation, characterised by extreme inflammation, fever, cytopaenias and organ dysfunction. HLH can be triggered by conditions such as infection, autoimmune disease and malignancy, among others. Both a familial and a secondary form have been described, the latter being increasingly recognised in adult patients with critical illness. HLH is difficult to diagnose, often under-recognised and carries a high mortality. Patients can present in a very similar fashion to sepsis and the two syndromes can co-exist and overlap, yet HLH requires specific immunosuppressive therapy. HLH should be actively excluded in patients with presumed sepsis who either lack a clear focus of infection or who are not responding to energetic infection management. Elevated serum ferritin is a key biomarker that may indicate the need for further investigations for HLH and can guide treatment. Early diagnosis and a multidisciplinary approach to HLH management may save lives.

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