Non-invasive Imaging of Oxygen Extraction Fraction in Adults with Sickle Cell Anaemia
Overview
Authors
Affiliations
Sickle cell anaemia is a monogenetic disorder with a high incidence of stroke. While stroke screening procedures exist for children with sickle cell anaemia, no accepted screening procedures exist for assessing stroke risk in adults. The purpose of this study is to use novel magnetic resonance imaging methods to evaluate physiological relationships between oxygen extraction fraction, cerebral blood flow, and clinical markers of cerebrovascular impairment in adults with sickle cell anaemia. The specific goal is to determine to what extent elevated oxygen extraction fraction may be uniquely present in patients with higher levels of clinical impairment and therefore may represent a candidate biomarker of stroke risk. Neurological evaluation, structural imaging, and the non-invasive T2-relaxation-under-spin-tagging magnetic resonance imaging method were applied in sickle cell anaemia (n = 34) and healthy race-matched control (n = 11) volunteers without sickle cell trait to assess whole-brain oxygen extraction fraction, cerebral blood flow, degree of vasculopathy, severity of anaemia, and presence of prior infarct; findings were interpreted in the context of physiological models. Cerebral blood flow and oxygen extraction fraction were elevated (P < 0.05) in participants with sickle cell anaemia (n = 27) not receiving monthly blood transfusions (interquartile range cerebral blood flow = 46.2-56.8 ml/100 g/min; oxygen extraction fraction = 0.39-0.50) relative to controls (interquartile range cerebral blood flow = 40.8-46.3 ml/100 g/min; oxygen extraction fraction = 0.33-0.38). Oxygen extraction fraction (P < 0.0001) but not cerebral blood flow was increased in participants with higher levels of clinical impairment. These data provide support for T2-relaxation-under-spin-tagging being able to quickly and non-invasively detect elevated oxygen extraction fraction in individuals with sickle cell anaemia with higher levels of clinical impairment. Our results support the premise that magnetic resonance imaging-based assessment of elevated oxygen extraction fraction might be a viable screening tool for evaluating stroke risk in adults with sickle cell anaemia.
Brain Age Modeling and Cognitive Outcomes in Young Adults With and Without Sickle Cell Anemia.
Ford A, Fellah S, Wang Y, Unger-Levinson K, Hagan M, Reis M JAMA Netw Open. 2025; 8(1):e2453669.
PMID: 39821401 PMC: 11742535. DOI: 10.1001/jamanetworkopen.2024.53669.
Afzali-Hashemi L, Baas K, Schrantee A, Nur E, Vu C, Choi S Hemasphere. 2024; 8(12):e70022.
PMID: 39624832 PMC: 11610621. DOI: 10.1002/hem3.70022.
Gassner G, Damestani N, Wheeler N, Kufer J, Yadav S, Mellen S J Cereb Blood Flow Metab. 2024; :271678X241300394.
PMID: 39568243 PMC: 11580122. DOI: 10.1177/0271678X241300394.
Cerebral vascular shunting and oxygen metabolism in sickle cell disease.
Song A, Richerson W, Richerson W, Aumann M, Aumann M, Waddle S Blood Adv. 2024; 9(2):386-397.
PMID: 39546745 PMC: 11787477. DOI: 10.1182/bloodadvances.2024014201.
Wang Y, Fellah S, Reis M, Guilliams K, Fields M, Steger-May K Neurology. 2024; 103(11):e210032.
PMID: 39546738 PMC: 11573263. DOI: 10.1212/WNL.0000000000210032.