Hematopoietic Stem Cell Transplant
Children with sickle cell disease (SCD) who received a hematopoietic stem cell transplant (HSCT) showed enhanced blood flow and oxygen extraction percentage in the brain, a measure of how well oxygen is utilised by tissues.
These results, according to experts, may help to explain why HSCT in SCD patients lowers the incidence of silent strokes or silent cerebral infarctions, both of which are recurrent strokes. Children who have sickle cell anaemia are more likely to experience silent cerebral infarctions, which are strokes that don't manifest any obvious symptoms.
Compared to prolonged transfusion therapy, which is frequently used to treat sickle cell disease, stem cell transplant may restore "brain" oxygen delivery reserve, offering higher stroke protection, the authors stated.
A uncommon blood illness called SCD causes crescent-shaped red blood cells that clog and damage blood arteries, increasing the risk of consequences including strokes.
Compared to healthy children, children with SCD are more likely to suffer from silent cerebral infarctions and strokes. Like a stroke, a silent cerebral infarction may produce cognitive deficits, such as issues in thinking and learning.
Treatments for kids with SCD
Chronic transfusions of red blood cells can lower the risk of both initial and recurrent strokes. However, this therapy results in second strokes in up to 20% of children and new or more severe silent cerebral infarctions in up to 25% of children.
According to studies, patients who receive HSCT as opposed to chronic red blood cell transfusion therapy had a lower risk of having another stroke or silent cerebral infarction. Red blood cell progenitor cells are changed as part of the HSCT procedure. It is currently the only possibly curative treatment for SCD that is available.
In the brain areas with the lowest cerebral blood flow in SCD children, the oxygen extraction fraction, or OEF, is highest. Additionally, these are the areas where quiet cerebral infarctions are most likely to occur.
The absence of red blood cells, which is also a typical symptom of SCD, triggers compensatory mechanisms in the brain that increase blood flow and OEF, according to researchers. Additionally, compared to healthy and anaemic controls, adults and kids with SCD are said to have increased cerebral blood flow.
While some children and adults with sickle cell disease have showed improved cerebral blood flow and OEF after receiving HSCT, it is yet unknown whether these individuals have levels that are comparable to those seen in healthy controls.
After getting a transplant, a group of American researchers wanted to investigate how HSCT affected the brains of kids with sickle cell disease. The researchers also looked into whether HSCT would be more efficient than chronic red blood cell transfusion therapy at reducing brain blood flow and OEF.
Ten SCD patients who were scheduled to have an HSCT were included in their 2015–2019 study. Four of the participants were female, and the participants' ages ranged from 9.5 to 16.7. 20 SCD patients' siblings and 20 SCD patients receiving chronic red blood cell transfusion therapy were also included by the researchers.
MRI scans were used to quantify brain blood flow and OEF before, three months before, and one to two years after HSCT.
The most prevalent form of SCD, sickle cell anaemia, was seen in seven of the individuals in the HSCT group. Three of them had sickle cell beta thalassemia, a more severe form of SCD. Blood cell precursors were acquired from a matched sibling donor in seven individuals and from a matched unrelated donor in three patients.
Three of the 10 kids had strokes before receiving HSCT, and five had silent cerebral infarctions. One's transcranial Doppler ultrasound velocities were peculiar. They were all highly susceptible to getting an early stroke.
Prior to the course of treatment, the HSCT group exhibited greater levels of brain blood flow than the controls (93.5 vs. 69.8 mL/100g/min). These levels decreased to 72.7 ml/100 g/min following HSCT.
Prior to HSCT, OEF was similarly higher compared to controls (36.8% vs. 30.9%), but after HSCT, it was discovered to be lower (27.0%).
Furthermore, HSCT patients experienced a greater post-HSCT drop in cerebral blood flow and OEF compared to kids who received chronic red blood cell transfusion therapy following a planned transfusion.
The researchers reported that after receiving a stem cell transplant, "Children with SCD showed stabilisation of cerebral hemodynamics, becoming identical to control children."
Future research should concentrate on the impact of stem cell source, preparative regimen, and recipient age on [factors that alter brain blood flow] in order to increase the proportion of children with SCD who can receive curative therapy in a way that maximises neuroprotection.
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