Oxygen gradient ektacytometry does not predict pain in children with sickle cell anaemia

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The loss of red blood cell (RBC) deformability in sickle cell anaemia (SCA) is considered the primary factor responsible for episodes of acute pain and downstream progressive organ dysfunction. Oxygen gradient ektacytometry (Oxygenscan) is a recently commercialised functional assay that aims to describe the deformability of RBCs in SCA at differing oxygen tensions. So far, the Oxygenscan has been evaluated only by a small number of research groups and the validity and clinical value of Oxygenscan-derived biomarkers have not yet been fully established. In this study we examined RBC deformability measured with the Oxygenscan in 91 children with SCA at King’s College Hospital in London. We found a significant correlation between Oxygenscan-derived biomarkers and well-recognised modifiers of disease severity in SCA: haemoglobin F and co-inherited α-thalassaemia. We failed, however, to find any independent predictive value of the Oxygenscan in the clinical outcome measure of pain, as well as other important parameters such as hydroxycarbamide treatment. Although the Oxygenscan remains an intriguing tool for basic research, our results question whether it provides any additional information in predicting the clinical course in children with SCA, beyond measuring known markers of disease severity.

OriginalsprogEngelsk
TidsskriftBritish Journal of Haematology
Vol/bind197
Sider (fra-til)609-617
ISSN0007-1048
DOI
StatusUdgivet - 2022

Bibliografisk note

Funding Information:
This research was funded in whole, or in part, by the Wellcome Trust (202800/Z/16/Z and 091758/Z/10/Z). For the purpose of open access, the author has applied a CC BY public copyright licence to any Author Accepted Manuscript version arising from this submission.

Funding Information:
The study was funded by the Danish Centre for Haemoglobinopathies, Copenhagen University Hospital (Rigshospitalet), Copenhagen, Denmark. Thomas N. Williams was funded through senior research fellowships from the Wellcome Trust (202800/Z/16/Z and 091758/Z/10/Z) who also provide core support to the KEMRI‐Wellcome Trust Research Programme in Kilifi, Kenya (203077/Z/16/Z).

Publisher Copyright:
© 2021 The Authors. British Journal of Haematology published by British Society for Haematology and John Wiley & Sons Ltd.

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