From the Departments of Radiology (M.T.W., A.S., E.S.M., Z.P.K.)
George Washington University Hospital (M.T.W., J.W., Z.P.K., J.L.C., A.A.), Washington, DC.
AJNR Am J Neuroradiol. 2018 Nov;39(11):2132-2139. doi: 10.3174/ajnr.A5830. Epub 2018 Oct 11.
Hematopoietic marrow hyperplasia and hyperperfusion are compensatory mechanisms in sickle cell anemia. We have observed marrow diffusion and arterial spin-labeling perfusion changes in sickle cell anemia following bone marrow transplantation. We aimed to compare arterial spin-labeling perfusion and marrow diffusion/ADC values in patients with sickle cell anemia before and after bone marrow transplantation or transfusion.
We reviewed brain MRIs from patients with sickle cell anemia obtained during 6 consecutive years at a children's hospital. Quantitative marrow diffusion values were procured from the occipital and sphenoid bones. Pseudocontinuous arterial spin-labeling perfusion values (milliliters/100 g of tissue/min) of MCA, anterior cerebral artery, and posterior cerebral artery territories were determined. Territorial CBF, whole-brain average CBF, and marrow ADC values were compared for changes before and after either bone marrow transplantation or transfusion. Bone marrow transplantation and transfusion groups were compared. Two-tailed paired and unpaired Student tests were used; < .05 was considered significant.
Fifty-three examinations from 17 patients with bone marrow transplantation and 29 examinations from 9 patients with transfusion were included. ADC values significantly increased in the sphenoid and occipital marrow following bone marrow transplantation in contrast to patients with transfusion ( > .83). Whole-brain mean CBF significantly decreased following bone marrow transplantation (77.39 ± 13.78 to 60.39 ± 13.62 ml/100 g tissue/min; < .001), without significant change thereafter. CBF did not significantly change following the first (81.11 ± 12.23 to 80.25 ± 8.27 ml/100 g tissue/min; = .47) or subsequent transfusions. There was no significant difference in mean CBF between groups before intervention ( = .22).
Improved CBF and marrow diffusion eventuate following bone marrow transplantation in children with sickle cell anemia in contrast to transfusion therapy.
造血骨髓增生和充血是镰状细胞贫血的代偿机制。我们观察到骨髓移植后镰状细胞贫血患者的骨髓弥散和动脉自旋标记灌注变化。我们旨在比较镰状细胞贫血患者骨髓移植或输血前后的动脉自旋标记灌注和骨髓弥散/ADC 值。
我们回顾了 6 年来在一家儿童医院获得的镰状细胞贫血患者的脑 MRI。从枕骨和蝶骨获取定量骨髓弥散值。确定 MCA、大脑前动脉和大脑后动脉区域的伪连续动脉自旋标记灌注值（毫升/100 克组织/分钟）。比较骨髓移植或输血前后各脑区 CBF、全脑平均 CBF 和骨髓 ADC 值的变化。比较骨髓移植组和输血组。使用双侧配对和非配对学生 t 检验；<.05 被认为有统计学意义。
纳入 17 例骨髓移植患者的 53 次检查和 9 例输血患者的 29 次检查。与输血患者相比，骨髓移植后蝶骨和枕骨骨髓的 ADC 值显著增加（>.83）。骨髓移植后全脑平均 CBF 显著降低（77.39±13.78 至 60.39±13.62ml/100g 组织/min；<.001），此后无显著变化。首次（81.11±12.23 至 80.25±8.27ml/100g 组织/min；=0.47）或随后的输血后 CBF 均无显著变化。干预前两组间平均 CBF 无显著差异（=0.22）。
与输血治疗相比，骨髓移植后儿童镰状细胞贫血患者的 CBF 和骨髓弥散得到改善。
