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Patients with XLP type 1 have variable numbers of NKT cells

Ralph, Elizabeth; Evans, Josie; Booth, Claire; Gilmour, Kimberly; (2022) Patients with XLP type 1 have variable numbers of NKT cells. British Journal of Haematology , 198 (1) pp. 151-154. 10.1111/bjh.18159. Green open access

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Abstract

X-linked lymphoproliferative disease (XLP1) is a rare primary immunodeficiency that usually presents in early childhood. Patients with XLP1 have been reported to have absent NKT cells, and it has been suggested that this can be diagnostic for the disorder. Whilst NKT frequency in adults is variable, little is known about their frequency in children. Therefore, we established a paediatric reference range for these cells. In contrast to previous reports, in our cohort of XLP1 patients, NKT cell numbers were found to be variable, and we would advise against using the finding of NKT cells to exclude a diagnosis of XLP1.

Type: Article
Title: Patients with XLP type 1 have variable numbers of NKT cells
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.1111/bjh.18159
Publisher version: https://doi.org/10.1111/bjh.18159
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher's terms and conditions.
Keywords: laboratory, lymphoproliferative disease, NKT cells
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Infection, Immunity and Inflammation Dept
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
URI: https://discovery.ucl.ac.uk/id/eprint/10155064
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