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

Cox, Miriam F.; Mackenzie, Strachan; Low, Ryan; Brown, Michael; Sanchez, Emilie; Carr, Aisling; Carpenter, Ben; Bishton, Mark; Duncombe, Andrew; Akpabio, Akpabio; Kulasekararaj, Austin; Sin, Fang En; Jones, Alexis; Kavirayani, Akhila; Sen, Ethan S.; Quick, Vanessa; Dulay, Gurdeep S.; Clark, Sam; Bauchmuller, Kris; Tattersall, Rachel S.; Manson, Jessica J.

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


Authors

Miriam F. Cox

Strachan Mackenzie

Ryan Low

Michael Brown

Emilie Sanchez

Aisling Carr

Ben Carpenter

Mark Bishton

Andrew Duncombe

Akpabio Akpabio

Austin Kulasekararaj

Fang En Sin

Alexis Jones

Akhila Kavirayani

Ethan S. Sen

Vanessa Quick

Gurdeep S. Dulay

Sam Clark

Kris Bauchmuller

Rachel S. Tattersall

Jessica J. Manson



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.

Journal Article Type Article
Acceptance Date Oct 1, 2023
Online Publication Date Nov 29, 2023
Publication Date Jan 1, 2024
Deposit Date Nov 30, 2023
Publicly Available Date May 30, 2024
Journal The Lancet Rheumatology
Electronic ISSN 2665-9913
Publisher Elsevier
Peer Reviewed Peer Reviewed
Volume 6
Issue 1
Pages e51-e62
DOI https://doi.org/10.1016/s2665-9913%2823%2900273-4
Keywords Immunology; Immunology and Allergy; Rheumatology
Public URL https://nottingham-repository.worktribe.com/output/27867585
Publisher URL https://www.thelancet.com/journals/lanrhe/article/PIIS2665-9913(23)00273-4/fulltext
Related Public URLs https://www.sciencedirect.com/science/article/abs/pii/S2665991323002734?via%3Dihub