Cerebral Arteriopathy Risk More Common in Sickle Cell Anemic Children


The sickle cell disease subtype is associated with increased risk among patients as young as 2 years old.

sickle cell anemia

Children with sickle cell anemia face an increased risk of intracranial and extracranial arteriopathy from as early an age as 2 years old, according to newly published findings.

In data presented at the American Society of Hematology (ASH) 2020 Annual Meeting this week, a team of investigators from Paris, France reported a greater prevalence of arteriopathy among pediatric patients with sickle cell anemia versus those with other hemoglobin subtypes.

Led by Francoise Bernaudin, MD, from the CHIC Hospital at University Paris XII, investigators sought to evaluated and compare cumulative intracranial and extracranial arteriopathy incidence and the associated risk factors from a longitudinal cohort database.

As they noted, cerebral vasculopathy drives overt strokes and silent cerebral infarcts (SCI). The team previously found that internal carotid artery (eICA) intracranial time averaged mean velocities (TAMV) ≥160 cm/s is very predictive of eICA stenosis, and an observed risk factor for SCI regardless of acute and chronic anemia.

“However, the kinetics of eICA arteriopathy are unknown,” they wrote.

Bernaudin and colleagues observed children born between 1988-2018 who were followed at their care center until at least June 2012 and up to September 2019. They annually assessed patients via Transcranial Doppler (TCD) imaging as well as at lease 1 cervical Doppler test.

The assessment overall included 493 patients with sickle cell disease, with 398 being anemic and 95 being sickle cell beta thalassemia (Sb).

Mean baseline biologic parameters were recorded between patients’ 1-3 years of age, a minimum of 3 months post-transfusion, 1 month post-episode of pain, and before any intensive therapy. Investigators recorded patients’ alpha-genes, b-globin haplotypes, G6PD activity, CD36 expression.

Median patient follow-up was 10.6 years, totaling 5335 patient-years. Among all patients, 6 deaths occurred, and 3 patients suffered an ischemic stroke.

Among children with sickle cell anemia, abnormal eICA TAMV and/or eICA stenosis were somewhat associated abnormal intracranial TAMV and/or stenosis. However, isolated eICA TAMV ≥200 cm/s or 160-199 cm/s were observed in 4.8% and 7.0% of all patients, respectively. Isolated eICA stenoses were observed in 11.2% of cases with such TAMV levels.

Intracranial stenosis was associated with a nearly four-fold increased likelihood of isolated intracranial TAMV ≥200 cm/s (HR, 4.25; 95% CI, 2.14 – 8.47; P <.001). When adjusted with this level of isolated intracranial TAMV, a-thalassemia, low hemoglobin, high WBC, MCV and LDH remained as significant risk factors for intracranial stenosis

“While we confirm that only SCA and not SC/Sb+ children are at risk of intra/extracranial arteriopathy, we show for the first time that extracranial arteriopathy progressively develops as early as 2 years old in SCA-children and reaches a plateau around 10 years of age, as for intracranial arteriopathy,” investigators wrote. “Furthermore, eICA tortuosities, which are the risk factor for eICA arteriopathy, are themselves significantly and independently associated with the SCA genotype and the severity of hemolytic anemia.”

The study, “Cumulative Incidences and Risk Factors for Intra and Extracranial Cerebral Arteriopathy in Sickle Cell Disease Children,” was presented at ASH 2020.

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