Children who undergo hematopoietic stem cell transplant (HSCT) for sickle cell disease (SCD) experience normalization of whole-brain hemodynamics, according to a cohort study published in Blood.

“This restoration of cerebral oxygen reserve may explain stroke protection following HSCT in this high-risk patient population,” the authors wrote in their report.

Children with SCD are at high-risk of experiencing stroke or silent cerebral infarctions. Although the reason for these events are not completely understood, it is hypothesized that cerebral hemodynamic stress may be involved. The aim of this study was to determine if HSCT normalizes cerebral hemodynamics.


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This prospective, single-center, cohort study included 10 children with SCD, 20 children who received chronic red blood cell transfusion therapy (CRTT), and 20 siblings of SCD patients who underwent MRI to measure whole-brain cerebral blood flow (CBF) and oxygen extraction fraction (OEF) 1 to 3 months prior to HSCT and 12 to 24 months after HSCT. Of the children with SCD included in the study, 3 had experience prior overt strokes, 5 had a silent stroke, and 1 had abnormal transcranial Doppler ultrasound velocities.

HSCT resulted in improved cerebral hemodynamics. CBF decreased from 93.5 mL/100 g/min prior to transplant to 72.7 mL/100 g/min (P =.004) and OEF decreased from 36.8% to 27.0% (P =.002). The CBF and OEF before and after HSCT were similar among children without SCD.

Children with SCD who underwent HSCT demonstrated greater improvement in CBF and OEF compared with children with SCD who instead received CRTT. For children who underwent HSCT, the reduction in CBF was -19.4 mL/100 g/min and was -8.1% in OEF compared with -0.9 mL/100 g/min (P =.024) and -3.3% (P =.001), respectively, among children who received a transfusion of CRTT.

The authors concluded that, “we demonstrate that cerebral hemodynamic and metabolic stress are relieved by successful HSCT in children with SCD, and CBF and OEF normalize following HSCT.”

Disclosures: Some of the study authors declared affiliations with biotech, pharmaceutical, and/or device companies. Please see the original reference for a full list of disclosures.

Reference

Hulbert M, Fields M, Guilliams K, et al. Normalization of cerebral hemodynamics following hematopoietic stem cell transplant in children with sickle cell disease. Blood. Published online August 30, 2022. doi: 10.1182/blood.2022016618