Abstract
SummaryThe 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.
Highlights
Sickle haemoglobin is a structural variant of haemoglobin A (HbA)
In this study we aimed to investigate the relationship between Oxygenscan-derived biomarkers and two established markers of disease severity in sickle cell anaemia (SCA): haemoglobin F (HbF) and co-inherited a-thalassaemia
Results were similar when controlling for age, HU treatment, %HbF and a-thalassaemia genotype
Summary
Sickle haemoglobin (haemoglobin S, HbS) is a structural variant of haemoglobin A (HbA). Sickle cell disease (SCD) refers to any symptomatic condition caused by HbS polymerisation within red blood cells (RBCs), with the most common and severe condition, sickle cell anaemia (SCA), resulting from homozygosity for the sickle mutation (HbSS). SCA is characterised by recurrent episodes of acute illness, chronic haemolytic anaemia and organ damage, as well as a significant reduction in life expectancy.[1]. When the oxygen tension is low, HbS polymerises causing the RBCs to become rigid and distorted—so-called sickling. Sickled RBCs are less deformable in the microcirculation, causing microvascular occlusion and haemolysis. The loss of RBC deformability in SCA is considered the primary factor responsible for episodes of acute pain and downstream progressive organ dysfunction.[2,3] An increased number of dense a 2021 The Authors.
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
