Effect of voxelotor on cerebral perfusion and cerebral oxygen metabolism and cardiac stress in adult patients with sickle cell disease

Sickle cell disease (SCD) is complicated by silent cerebral infarcts (SCIs), for which anemia is an important risk factor. Despite normal oxygen delivery (OD), cerebral vascular reserve (CVR), and cerebral metabolic rate of oxygen (CMRO2) are diminished in SCD, possibly causing the formation of SCIs...

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Veröffentlicht in:American journal of hematology 2025-01, Vol.100 (1), p.78-84
Hauptverfasser: Konté, Kadère, Afzali‐Hashemi, Liza, Baas, Koen P. A., Schrantee, Anouk, Wood, John C., Nur, Erfan, Nederveen, Aart J., Biemond, Bart J.
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Sprache:eng
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Zusammenfassung:Sickle cell disease (SCD) is complicated by silent cerebral infarcts (SCIs), for which anemia is an important risk factor. Despite normal oxygen delivery (OD), cerebral vascular reserve (CVR), and cerebral metabolic rate of oxygen (CMRO2) are diminished in SCD, possibly causing the formation of SCIs. Voxelotor inhibits polymerization by increasing the hemoglobin oxygen binding, ameliorating hemolytic anemia. Furthermore, anemia is related to cardiac complications. Our aims were to assess the effect of voxelotor on markers of cerebral perfusion, cerebral oxygen metabolism, and markers of cardiac stress in SCD patients. Cerebral hemodynamics and oxygen metabolism were measured with MRI before and after 3 months of voxelotor treatment (1500 mg/day) in 18 adults with SCD (HbSS/HbSβ0‐thalassemia). Hemoglobin levels significantly increased (p = .001) and markers of hemolysis decreased (p 
ISSN:0361-8609
1096-8652
1096-8652
DOI:10.1002/ajh.27522