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Comment
. 2014 Aug 7;124(6):830-1.
doi: 10.1182/blood-2014-06-582403.

Understanding sickle cell brain drain

Affiliations
Comment

Understanding sickle cell brain drain

Monica L Hulbert et al. Blood. .

Abstract

In this issue of Blood, Helton et al highlight the neuroradiological findings of children enrolled in the Strokes With Transfusions Changing to Hydroxyurea (SWiTCH) trial. This study, which screened 161 children with sickle cell anemia (SCA) receiving chronic transfusion therapy for prevention of recurrent strokes, is the largest SCA cohort followed prospectively with magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) published to date, giving additional insights into the relationships between strokes and cerebral vasculopathy in SCA.

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Conflict of interest statement

Conflict-of-interest disclosure: The authors declare no competing financial interests.

Figures

None
Schematic representation of brain perfusion, oxygen extraction, and oxygen metabolism in children with SCA. (A) Proposed model of cerebral infarction in SCA with cerebral vasculopathy. CBF declines as vasculopathy progresses. The percentage of oxygen extracted from the blood by the brain (OEF) increases to maintain adequate tissue oxygen utilization (CMRO2). When CBF continues to fall and OEF cannot increase further, CMRO2 drops, causing cerebral infarction. (B) Proposed model of cerebral infarction in SCA without cerebral vasculopathy. CBF is elevated to compensate for decreased blood oxygen content and has limited additional capacity to increase. As blood oxygen content falls due to acute illness, OEF increases to maintain adequate CMRO2. When OEF is maximized, a drop in CBF may cause CMRO2 to fall, and infarction ensues. The figure has been adapted from Powers4 with permission.

Comment on

References

    1. Helton KJ, Adams RJ, Kesler KL, et al. Magnetic resonance imaging/angiography and transcranial Doppler velocities in sickle cell anemia: results from the SWiTCH trial. Blood. 2014;124(6):891–898. - PMC - PubMed
    1. Adams RJ, McKie VC, Carl EM, et al. Long-term stroke risk in children with sickle cell disease screened with transcranial Doppler. Ann Neurol. 1997;42(5):699–704. - PubMed
    1. Hulbert ML, McKinstry RC, Lacey JL, et al. Silent cerebral infarcts occur despite regular blood transfusion therapy after first strokes in children with sickle cell disease. Blood. 2011;117(3):772–779. - PMC - PubMed
    1. Powers WJ. Cerebral hemodynamics in ischemic cerebrovascular disease. Ann Neurol. 1991;29(3):231–240. - PubMed
    1. Prohovnik I, Hurlet-Jensen A, Adams R, De Vivo D, Pavlakis SG. Hemodynamic etiology of elevated flow velocity and stroke in sickle-cell disease. J Cereb Blood Flow Metab. 2009;29(4):803–810. - PubMed