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A validation study of the identification of haemophagocytic lymphohistiocytosis in England using population-based health data

Lookup NU author(s): Professor Judith Rankin, Dr Ethan SenORCiD


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© 2021 British Society for Haematology and John Wiley & Sons Ltd.We assessed the validity of coded healthcare data to identify cases of haemophagocytic lymphohistiocytosis (HLH). Hospital Episode Statistics (HES) identified 127 cases within five hospital Trusts 2013–2018 using ICD-10 codes D76.1, D76.2 and D76.3. Hospital records were reviewed to validate diagnoses. Out of 74 patients, 73 were coded D76.1 or D76.2 (positive predictive value 89·0% [95% Confidence Interval {CI} 80·2–94·9%]) with confirmed/probable HLH. For cases considered not HLH, 44/53 were coded D76.3 (negative predictive value 97·8% [95% CI 88·2–99·9%]). D76.1 or D76.2 had 68% sensitivity in detecting HLH compared to an established active case-finding HLH register in Sheffield. Office for National Statistics (ONS) mortality data (2003–2018) identified 698 patients coded D76.1, D76.2 and D76.3 on death certificates. Five hundred and forty-one were coded D76.1 or D76.2 of whom 524 (96·9%) had HLH in the free-text cause of death. Of 157 coded D76.3, 66 (42·0%) had HLH in free text. D76.1 and D76.2 codes reliably identify HLH cases, and provide a lower bound on incidence. Non-concordance between D76.3 and HLH excludes D76.3 as an ascertainment source from HES. Our results suggest electronic healthcare data in England can enable population-wide registration and analysis of HLH for future research.

Publication metadata

Author(s): Bishton MJ, Stilwell P, Card TR, Lanyon P, Ban L, Elliss-Brookes L, Manson J, Nanduri V, Earp K, Flower L, Amarnani R, Rankin J, Sen ES, Tattersall RS, Crooks CJ, Aston J, Siskova V, West J, Bythell M

Publication type: Article

Publication status: Published

Journal: British Journal of Haematology

Year: 2021

Volume: 194

Issue: 6

Pages: 1039-1044

Print publication date: 01/09/2021

Online publication date: 12/08/2021

Acceptance date: 28/07/2021

ISSN (print): 0007-1048

ISSN (electronic): 1365-2141

Publisher: John Wiley and Sons Inc


DOI: 10.1111/bjh.17768


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