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Lookup NU author(s): Dr Ethan SenORCiD
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© 2024 Elsevier LtdHaemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome characterised by persistently activated cytotoxic lymphocytes and macrophages, which, if untreated, leads to multiorgan dysfunction and death. HLH should be considered in any acutely unwell patient not responding to treatment as expected, with prompt assessment to look for what we term the three Fs—fever, falling blood counts, and raised ferritin. Worldwide, awareness of HLH and access to expert management remain inequitable. Terminology is not standardised, classification criteria are validated in specific patient groups only, and some guidelines rely on specialised and somewhat inaccessible tests. The consensus guideline described in this Health Policy was produced by a self-nominated working group from the UK network Hyperinflammation and HLH Across Speciality Collaboration (HiHASC), a multidisciplinary group of clinicians experienced in managing people with HLH. Combining literature review and experience gained from looking after patients with HLH, it provides a practical, structured approach for all health-care teams managing adult (>16 years) patients with possible HLH. The focus is on early recognition and diagnosis of HLH and parallel identification of the underlying cause. To ensure wide applicability, the use of inexpensive, readily available tests is prioritised, but the role of specialist investigations and their interpretation is also addressed.
Author(s): Cox MF, Mackenzie S, Low R, Brown M, Sanchez E, Carr A, Carpenter B, Bishton M, Duncombe A, Akpabio A, Kulasekararaj A, Sin FE, Jones A, Kavirayani A, Sen ES, Quick V, Dulay GS, Clark S, Bauchmuller K, Tattersall RS, Manson JJ
Publication type: Review
Publication status: Published
Journal: The Lancet Rheumatology
Year: 2024
Volume: 6
Issue: 1
Pages: e51-e62
Print publication date: 01/01/2024
Online publication date: 29/11/2023
Acceptance date: 02/04/2018
ISSN (electronic): 2665-9913
Publisher: Elsevier Ltd
URL: https://doi.org/10.1016/S2665-9913(23)00273-4
DOI: 10.1016/S2665-9913(23)00273-4