Haemophagocytic lymphohistiocytosis in adult critical care
File(s)
Accepted version
Author(s)
Type
Journal Article
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, auto-immune disease and malignancy, amongst 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 underrecognised 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.
characterised by extreme inflammation, fever, cytopaenias and organ dysfunction. HLH can be
triggered by conditions such as infection, auto-immune disease and malignancy, amongst 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 underrecognised 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.
Date Issued
2020-08-01
Date Acceptance
2019-11-01
Citation
Journal of the Intensive Care Society, 2020, 21 (3), pp.256-268
ISSN
1751-1437
Publisher
SAGE Publications (UK and US)
Start Page
256
End Page
268
Journal / Book Title
Journal of the Intensive Care Society
Volume
21
Issue
3
Copyright Statement
The Intensive Care Society 2020. The final, definitive version of this paper has been published in Journal of the Intensive Care Society by Sage Publications Ltd. All rights reserved. It is available at: https://journals.sagepub.com/doi/10.1177/1751143719893865
Publication Status
Published
Date Publish Online
2020-01-07