White Matter Hyperintensity May Serve as Biomarker for SCD, Study Says

An analysis of white matter hyperintensity (WMH, a measure of the risk of stroke) may serve as a biomarker to track the progression of blood vessel anomalies associated with sickle cell disease (SCD) and to check whether treatments were effective, according to new research.

The research study, “Enhanced Long-Term Brain MRI Evaluation Of Children With Sickle Cell Disease Following Hematopoietic Cell Transplantation,” was published in the journal Biology of Blood and Marrow Transplantation.

SCD is associated with stroke in children due to progressive neurovasculopathy (damage in the blood vessels) affecting cognitive function and quality of life. However, hematopoietic cell transplant  (HCT) is believed to effectively stop the progression of neurovasculopathy, as measured by studies assessing the brain using magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA).

These techniques, however, do not provide much information on the status of smaller blood vessels, which can also be damaged in SCD children.

Another type of MRI, called FLAIR MRI, measures white matter hyperintensity and can detect anomalies in small blood vessels. In SCD children, the larger the WMH volume, the higher the decrease in cognitive function.

To investigate whether analysis of WMH could complement MRI assessments of brain blood vessels before and after HCT therapy in children, researchers analyzed scheduled annual MRI/MRA and WMH analyses performed before and after HTC at scheduled intervals in 18 SCD children with a mean age of 9. They also measured quality of life in these patients.

The analysis indicated that MRIs done before the treatment showed that nine and five patients had sickle-related stroke and/or small infarcts, respectively, whereas only four patients had a normal scan.

Researchers then divided patients according to the severity of WMH, determined by analysis of pre-treatment WMH volume, and assessed both the MRI and WMH data on these patients one to seven years after HTC treatment. They saw that MRI/MRA and WMH volume were stable or slightly improved in nearly all patients (17 of 18), showing that HTC treatment had worked.

Also, quality of life was significantly improved after HCT treatment in children in the lowest WMH group compared to the other groups, as reported by both patients and parents.

“WMH appears to quantitatively support MRI-based findings that HCT [treatment] stabilizes long-term small and large vessel  cerebrovascular changes, and is associated with the degree of  improved [quality of life],” researchers concluded.

“While confirmation in larger prospective studies and evaluation by  neurocognitive testing are needed, these findings suggest that WMH is a useful biomarker of neurovasculopathy post-transplant for SCD,” they wrote.