Cerebral hemodynamics and oxygen metabolism in patients with milder and severe forms of sickle cell disease and thalassemia
Abstract Silent cerebral infarcts (SCIs) are present in patients with sickle cell disease (SCD) and thalassemia, but the pathophysiology of SCIs is not fully understood. Previous studies mainly focused on cerebral hemodynamics and oxygen metabolism in patients with severe SCD (HbSS/HbSβ°) but not in...
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Wiley
2024-12-01
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| Series: | HemaSphere |
| Online Access: | https://doi.org/10.1002/hem3.70022 |
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| author | Liza Afzali‐Hashemi Koen P. A. Baas Anouk Schrantee Erfan Nur Chau Vu Soyoung Choi Silvie Suriany John C. Wood Aart J. Nederveen Bart J. Biemond |
| author_facet | Liza Afzali‐Hashemi Koen P. A. Baas Anouk Schrantee Erfan Nur Chau Vu Soyoung Choi Silvie Suriany John C. Wood Aart J. Nederveen Bart J. Biemond |
| author_sort | Liza Afzali‐Hashemi |
| collection | DOAJ |
| description | Abstract Silent cerebral infarcts (SCIs) are present in patients with sickle cell disease (SCD) and thalassemia, but the pathophysiology of SCIs is not fully understood. Previous studies mainly focused on cerebral hemodynamics and oxygen metabolism in patients with severe SCD (HbSS/HbSβ°) but not in milder forms of SCD (HbSC/HbSβ+) and thalassemia despite the high prevalence of SCIs in these patients. In this work, we studied the cerebral hemodynamics and oxygen metabolism, and SCI lesion load in 75 severe and 26 mild adult SCD patients, 18 thalassemia patients (as anemic comparison group), and 30 healthy controls before and after a vasodilatory challenge with acetazolamide. Cerebral blood flow was significantly higher in patients with severe SCD and thalassemia compared to patients with mild SCD and controls (p < 0.05). Conversely, oxygen extraction fraction and cerebral metabolic rate of oxygen (CMRO2) were significantly lower in patients with severe SCD and thalassemia compared to other groups (p < 0.01). In contrast, no difference in SCI volumes was found between mild and severe SCD and thalassemia patients. After acetazolamide administration, oxygen delivery increased less in severe SCD and thalassemia patients compared to other groups (p < 0.01) and CMRO2 decreased only in severe SCD patients (p < 0.01). Given the reduced CMRO2 values in severe SCD and thalassemia patients, we conclude that reduced cerebral oxygen consumption in these patient groups is mostly related to anemia. Our data suggest that the pathophysiology of SCIs in patients with milder forms of SCD might be more related to prior episodes of anemia or other sickle cell‐related factors. |
| format | Article |
| id | doaj-art-ccbe48783dd54f508abc55ab5cffc9f6 |
| institution | Kabale University |
| issn | 2572-9241 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | Wiley |
| record_format | Article |
| series | HemaSphere |
| spelling | doaj-art-ccbe48783dd54f508abc55ab5cffc9f62025-01-07T12:35:28ZengWileyHemaSphere2572-92412024-12-01812n/an/a10.1002/hem3.70022Cerebral hemodynamics and oxygen metabolism in patients with milder and severe forms of sickle cell disease and thalassemiaLiza Afzali‐Hashemi0Koen P. A. Baas1Anouk Schrantee2Erfan Nur3Chau Vu4Soyoung Choi5Silvie Suriany6John C. Wood7Aart J. Nederveen8Bart J. Biemond9Department of Radiology & Nuclear Medicine Amsterdam University Medical Centers, University of Amsterdam Amsterdam The NetherlandsDepartment of Radiology & Nuclear Medicine Amsterdam University Medical Centers, University of Amsterdam Amsterdam The NetherlandsDepartment of Radiology & Nuclear Medicine Amsterdam University Medical Centers, University of Amsterdam Amsterdam The NetherlandsDepartment of Hematology Amsterdam University Medical Centers, University of Amsterdam Amsterdam The NetherlandsDepartment of Biomedical Engineering University of Southern California Los Angeles California USANeuroscience Graduate Program University of Southern California Los Angeles California USADivision of Cardiology Children's Hospital Los Angeles, Keck School of Medicine, University of Southern California Los Angeles California USADivision of Cardiology Children's Hospital Los Angeles, Keck School of Medicine, University of Southern California Los Angeles California USADepartment of Radiology & Nuclear Medicine Amsterdam University Medical Centers, University of Amsterdam Amsterdam The NetherlandsDepartment of Hematology Amsterdam University Medical Centers, University of Amsterdam Amsterdam The NetherlandsAbstract Silent cerebral infarcts (SCIs) are present in patients with sickle cell disease (SCD) and thalassemia, but the pathophysiology of SCIs is not fully understood. Previous studies mainly focused on cerebral hemodynamics and oxygen metabolism in patients with severe SCD (HbSS/HbSβ°) but not in milder forms of SCD (HbSC/HbSβ+) and thalassemia despite the high prevalence of SCIs in these patients. In this work, we studied the cerebral hemodynamics and oxygen metabolism, and SCI lesion load in 75 severe and 26 mild adult SCD patients, 18 thalassemia patients (as anemic comparison group), and 30 healthy controls before and after a vasodilatory challenge with acetazolamide. Cerebral blood flow was significantly higher in patients with severe SCD and thalassemia compared to patients with mild SCD and controls (p < 0.05). Conversely, oxygen extraction fraction and cerebral metabolic rate of oxygen (CMRO2) were significantly lower in patients with severe SCD and thalassemia compared to other groups (p < 0.01). In contrast, no difference in SCI volumes was found between mild and severe SCD and thalassemia patients. After acetazolamide administration, oxygen delivery increased less in severe SCD and thalassemia patients compared to other groups (p < 0.01) and CMRO2 decreased only in severe SCD patients (p < 0.01). Given the reduced CMRO2 values in severe SCD and thalassemia patients, we conclude that reduced cerebral oxygen consumption in these patient groups is mostly related to anemia. Our data suggest that the pathophysiology of SCIs in patients with milder forms of SCD might be more related to prior episodes of anemia or other sickle cell‐related factors.https://doi.org/10.1002/hem3.70022 |
| spellingShingle | Liza Afzali‐Hashemi Koen P. A. Baas Anouk Schrantee Erfan Nur Chau Vu Soyoung Choi Silvie Suriany John C. Wood Aart J. Nederveen Bart J. Biemond Cerebral hemodynamics and oxygen metabolism in patients with milder and severe forms of sickle cell disease and thalassemia HemaSphere |
| title | Cerebral hemodynamics and oxygen metabolism in patients with milder and severe forms of sickle cell disease and thalassemia |
| title_full | Cerebral hemodynamics and oxygen metabolism in patients with milder and severe forms of sickle cell disease and thalassemia |
| title_fullStr | Cerebral hemodynamics and oxygen metabolism in patients with milder and severe forms of sickle cell disease and thalassemia |
| title_full_unstemmed | Cerebral hemodynamics and oxygen metabolism in patients with milder and severe forms of sickle cell disease and thalassemia |
| title_short | Cerebral hemodynamics and oxygen metabolism in patients with milder and severe forms of sickle cell disease and thalassemia |
| title_sort | cerebral hemodynamics and oxygen metabolism in patients with milder and severe forms of sickle cell disease and thalassemia |
| url | https://doi.org/10.1002/hem3.70022 |
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