Hydroxycarbamide treatment in children with Sickle Cell Anaemia is associated with more intact white matter...

Area of Science:

• Neurology
• Hematology
• Medical Imaging

Background:

• Sickle cell anaemia (SCA) is a genetic blood disorder causing chronic anaemia, impaired cerebrovascular dilatory capacity, and cerebral infarctions.
• White matter (WM) integrity is crucial for brain function and can be affected in SCA.

Purpose of the Study:

• To assess the relationship between WM microstructural properties and cerebrovascular reactivity (CVR) and cerebral blood flow in SCA.
• To investigate the effects of hydroxycarbamide treatment on these relationships.

Main Methods:

• Utilized diffusion tensor imaging (DTI) to analyze white matter mean diffusivity (MD) histogram parameters (skew and kurtosis).
• Assessed cerebrovascular reactivity (CVR) in patients with SCA and healthy controls.
• Compared DTI and CVR metrics between untreated SCA patients, hydroxycarbamide-treated SCA patients, and healthy controls.

Main Results:

• Mean CVR was significantly increased in hydroxycarbamide-treated SCA patients compared to untreated patients.
• Untreated SCA patients exhibited increased WM MD histogram skew and kurtosis, indicating disrupted WM integrity.
• A significant linear relationship between CVR and WM MD histogram skew/kurtosis was observed in healthy controls but not in SCA patients.

Conclusions:

• Hydroxycarbamide treatment appears to improve CVR and normalize WM microstructural properties in SCA patients.
• WM MD histogram parameters in hydroxycarbamide-treated patients more closely resemble those of healthy controls.
• These findings highlight the potential of hydroxycarbamide in mitigating brain complications associated with SCA.