Abstract

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 t tests were used; P < .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 (P > .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; P < .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; P = .47) or subsequent transfusions. There was no significant difference in mean CBF between groups before intervention (P = .22). Improved CBF and marrow diffusion eventuate following bone marrow transplantation in children with sickle cell anemia in contrast to transfusion therapy.

Highlights

  • BACKGROUND AND PURPOSEHematopoietic marrow hyperplasia and hyperperfusion are compensatory mechanisms in sickle cell anemia

  • ADC values significantly increased in the sphenoid and occipital marrow following bone marrow transplantation in contrast to patients with transfusion (P ⬎ .83)

  • Arterial spin-labeling (ASL) perfusion images were evaluated qualitatively and quantitatively by a board-certified neuroradiologist with ⬎6 years of clinical experience after board certification who was blinded to the type of therapy/intervention at the time of assessment

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Summary

Objectives

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. The aim of this study was to compare and contrast alterations in ASL perfusion and bone marrow diffusion/

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