Diagnosis and investigation of suspected haemophagocytic lymphohistiocytosis in adults: 2023 Hyperinflammation and HLH Across Speciality Collaboration (HiHASC) consensus guideline.
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Authors
Cox, M.F
Mackenzie, S
Low, R
Brown, M
Sanchez, E
Carr, A
Carpenter, B
Bishton, , M
Duncombe, A
Akpabio, A
Issue Date
2024
Type
Published Abstract
Language
Keywords
Research Subject Categories::MEDICINE::Dermatology and venerology,clinical genetics, internal medicine::Internal medicine::Rheumatology
Alternative Title
Abstract
Abstract
Haemophagocytic 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.
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Conflict of interest statement
Declaration of interests AC reports payment or honoraria from CSL, Griffols, Akcea, Biogene, Neurodiem, and Bristol Myers Squibb. AC also reports honoraria from Lupin (£1000) and CSL (£1000) for advisory board participation and support from CSL for attending the Peripheral Nerve Society annual congress, Miami 2022. MBr reports membership of the Medicines and Healthcare products Regulatory Agency Commission of Human Medicines Expert Advisory Group and National Health Service England's Covid medicines Expert Working Group. MBi reports consulting fees from Lilly, Incyte, Roche, and Beigene; and payment or honoraria for lectures, presentations, speaker bureaus, manuscript writing, or educational events from Tevapharma and Celltrion. JJM reports funding from the UCLH Charity Grant funding given to the HLH Service to establish biobank and fund consultant time (£160 000 paid to UCLH). All other authors declare no competing interests.
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Publication types
Review
MeSH terms
Accidental Falls
Adult
Consensus
Ferritins
Humans
Lymphohistiocytosis, Hemophagocytic* / diagnosis
Macrophages
Substances
Ferritins
Related information
MedGen
PubChem Compound (MeSH Keyword)
LinkOut - more resources
Full Text Sources
Elsevier Science
Description
Citation
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; HiHASC group. Diagnosis and investigation of suspected haemophagocytic lymphohistiocytosis in adults: 2023 Hyperinflammation and HLH Across Speciality Collaboration (HiHASC) consensus guideline. Lancet Rheumatol. 2024 Jan;6(1):e51-e62. doi: 10.1016/S2665-9913(23)00273-4. Epub 2023 Nov 29. PMID: 38258680.