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Clinical Trial
. 2019 Apr;94(4):467-474.
doi: 10.1002/ajh.25423. Epub 2019 Feb 21.

White matter has impaired resting oxygen delivery in sickle cell patients

Yaqiong Chai  1 , Adam M Bush  2 , Julie Coloigner  3 , Aart J Nederveen  4 , Benita Tamrazi  5 , Chau Vu  1 , Soyoung Choi  6   7 , Thomas D Coates  8 , Natasha Lepore  1   5 , John C Wood  1   7
Affiliations
Clinical Trial

White matter has impaired resting oxygen delivery in sickle cell patients

Yaqiong Chai et al. Am J Hematol. 2019 Apr.

Abstract

Although modern medical management has lowered overt stroke occurrence in patients with sickle cell disease (SCD), progressive white matter (WM) damage remains common. It is known that cerebral blood flow (CBF) increases to compensate for anemia, but sufficiency of cerebral oxygen delivery, especially in the WM, has not been systematically investigated. Cerebral perfusion was measured by arterial spin labeling in 32 SCD patients (age range: 10-42 years old, 14 males, 7 with HbSC, 25 HbSS) and 25 age and race-matched healthy controls (age range: 15-45 years old, 10 males, 12 with HbAS, 13 HbAA); 8/24 SCD patients were receiving regular blood transfusions and 14/24 non-transfused SCD patients were taking hydroxyurea. Imaging data from control subjects were used to calculate maps for CBF and oxygen delivery in SCD patients and their T-score maps. Whole brain CBF was increased in SCD patients with a mean T-score of 0.5 and correlated with lactate dehydrogenase (r2 = 0.58, P < 0.0001). When corrected for oxygen content and arterial saturation, whole brain and gray matter (GM) oxygen delivery were normal in SCD, but WM oxygen delivery was 35% lower than in controls. Age and hematocrit were the strongest predictors for WM CBF and oxygen delivery in patients with SCD. There was spatial co-localization between regions of low oxygen delivery and WM hyperintensities on T2 FLAIR imaging. To conclude, oxygen delivery is preserved in the GM of SCD patients, but is decreased throughout the WM, particularly in areas prone to WM silent strokes.

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Conflict of interest statement

CONFLICT OF INTEREST

JCW receives research support in kind from Philips Healthcare. None of the other authors have conflicts relevant to the study.

Figures

FIGURE 1
FIGURE 1
White matter lesion mask overlaid on T-score map. The scaled WM lesion mask in its axial view is overlaid on top of the T-score for CBF (top row) and O2 delivery (bottom row). The number z indicates the coordinates in the standard atlas
FIGURE 2
FIGURE 2
WMH permutation results. The CBF and oxygen delivery distribution (top row) and T-score of CBF and oxygen delivery distribution (bottom row) in the normal appearing white matter (NAWM) and in the white matter hyperintensity (WMH) prone regions
See this image and copyright information in PMC

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