Haemophagocytic lymphohistiocytosis in adult critical care

Kris Bauchmuller; Jessica J Manson; Rachel Tattersall; Michael Brown; Christopher McNamara; Mervyn Singer; Stephen J Brett

doi:10.1177/1751143719893865

Haemophagocytic lymphohistiocytosis in adult critical care

J Intensive Care Soc. 2020 Aug;21(3):256-268. doi: 10.1177/1751143719893865. Epub 2020 Jan 7.

Authors

Kris Bauchmuller¹, Jessica J Manson², Rachel Tattersall^{3

4}, Michael Brown^{5

6}, Christopher McNamara⁷, Mervyn Singer⁸, Stephen J Brett⁹

Affiliations

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.

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.

Keywords: Lymphohistiocytosis; haemophagocytic; histiocytosis; macrophage activation syndrome; sepsis.