Diagnosis and investigation of suspected haemophagocytic lymphohistiocytosis in adults: 2023 Hyperinflammation and HLH Across Speciality Collaboration (HiHASC) consensus guideline.

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Cox, M.F
Mackenzie, S
Low, R
Brown, M
Sanchez, E
Carr, A
Carpenter, B
Bishton, , M
Duncombe, A
Akpabio, A
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2024
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Published Abstract
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Research Subject Categories::MEDICINE::Dermatology and venerology,clinical genetics, internal medicine::Internal medicine::Rheumatology
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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. Copyright © 2024 Elsevier Ltd. All rights reserved. PubMed Disclaimer 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. Similar articles The 2022 EULAR/ACR points to consider at the early stages of diagnosis and management of suspected haemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS). Shakoory B, Geerlinks A, Wilejto M, Kernan K, Hines M, Romano M, Piskin D, Ravelli A, Sinha R, Aletaha D, Allen C, Bassiri H, Behrens EM, Carcillo J, Carl L, Chatham W, Cohen JI, Cron RQ, Drewniak E, Grom AA, Henderson LA, Horne A, Jordan MB, Nichols KE, Schulert G, Vastert S, Demirkaya E, Goldbach-Mansky R, de Benedetti F, Marsh RA, Canna SW; HLH/MAS task force. Ann Rheum Dis. 2023 Oct;82(10):1271-1285. doi: 10.1136/ard-2023-224123. Epub 2023 Jul 24. PMID: 37487610 Free PMC article. The 2022 EULAR/ACR Points to Consider at the Early Stages of Diagnosis and Management of Suspected Haemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome (HLH/MAS). Shakoory B, Geerlinks A, Wilejto M, Kernan K, Hines M, Romano M, Piskin D, Ravelli A, Sinha R, Aletaha D, Allen C, Bassiri H, Behrens EM, Carcillo J, Carl L, Chatham W, Cohen JI, Cron RQ, Drewniak E, Grom AA, Henderson LA, Horne A, Jordan MB, Nichols KE, Schulert G, Vastert S, Demirkaya E, Goldbach-Mansky R, de Benedetti F, Marsh RA, Canna SW. Arthritis Rheumatol. 2023 Oct;75(10):1714-1732. doi: 10.1002/art.42636. Epub 2023 Jul 24. PMID: 37486733 Free PMC article. Diagnostic biomarkers for adult haemophagocytic lymphohistiocytosis in critically ill patients (HEMICU): a prospective observational study protocol. Lachmann G, Knaak C, von Haefen C, Paeschke N, Meisel C, Nyvlt P, Schuster FS, Piper SK, Kruppa J, Vorderwülbecke G, Balzer F, La Rosée P, Schenk T, Unterwalder N, Kölsch U, Lachmann N, Akyüz L, Brunkhorst FM, Volk HD, Keh D, Spies C. BMJ Open. 2019 Oct 30;9(10):e032695. doi: 10.1136/bmjopen-2019-032695. PMID: 31666276 Free PMC article. Review of haemophagocytic lymphohistiocytosis. Freeman HR, Ramanan AV. Arch Dis Child. 2011 Jul;96(7):688-93. doi: 10.1136/adc.2009.176610. Epub 2010 Jun 28. PMID: 20584844 Review. Diagnosing haemophagocytic syndrome. Sen ES, Steward CG, Ramanan AV. Arch Dis Child. 2017 Mar;102(3):279-284. doi: 10.1136/archdischild-2016-310772. Epub 2016 Oct 24. PMID: 27831908 Review. See all similar articles Cited by Secondary haemophagocytic lymphohistiocytosis: time for a united approach to diagnosis and cytokine blockade. Flower L, Manson JJ. Anaesth Rep. 2024 Apr 22;12(1):e12291. doi: 10.1002/anr3.12291. eCollection 2024 Jan-Jun. PMID: 38655565 No abstract available. Tuberculosis-Associated Hemophagocytic Lymphohistiocytosis: Diagnostic Challenges and Determinants of Outcome. Kurver L, Seers T, van Dorp S, van Crevel R, Pollara G, van Laarhoven A. Open Forum Infect Dis. 2024 Jan 11;11(4):ofad697. doi: 10.1093/ofid/ofad697. eCollection 2024 Apr. PMID: 38560612 Free PMC article. 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
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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.
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