Early detection and management of extracranial arteriopathy reduces the incidence of silent cerebral infarcts in sickle cell anemia: a long-term prospective cohort study
Previous reports about the Creteil newborn-cohort (1988/Apr-2007) showed that the risk of silent cerebral infarcts (SCI) remained high (37.1%) by age 14 in children with sickle cell anemia (SCA) and intracranial time-averaged mean maximum velocity (TAMMV)≥200cm/s despite chronictransfusion. Systema...
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Ferrata Storti Foundation
2025-06-01
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| Series: | Haematologica |
| Online Access: | https://haematologica.org/article/view/12140 |
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| author | Francoise Bernaudin Cecile Arnaud Annie Kamdem Jenny Youn Manuela Vasile Isabelle Hau Fouad Madhi Aline Malterre Celine Delestrain Ralph Epaud Camille Jung Suzanne Verlhac |
| author_facet | Francoise Bernaudin Cecile Arnaud Annie Kamdem Jenny Youn Manuela Vasile Isabelle Hau Fouad Madhi Aline Malterre Celine Delestrain Ralph Epaud Camille Jung Suzanne Verlhac |
| author_sort | Francoise Bernaudin |
| collection | DOAJ |
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Previous reports about the Creteil newborn-cohort (1988/Apr-2007) showed that the risk of silent cerebral infarcts (SCI) remained high (37.1%) by age 14 in children with sickle cell anemia (SCA) and intracranial time-averaged mean maximum velocity (TAMMV)≥200cm/s despite chronictransfusion. Systematic assessment of extracranial internal carotid artery (eICA) since June-2011 revealed that SCI-risk is associated with chronic or acute anemia and eICA-stenosis. Based on these results, SCA-children with eICA-TAMMV≥200cm/s or eICA-stenosis were placed on chronictransfusion and considered for allogeneic stem-cell transplantation (alloSCT). SCA-children with 160-199cm/s eICA-TAMMV were maintained on hydroxyurea. We hypothesized that detection/management of eICA-arteriopathy and wider use of hydroxyurea could reduce SCI-incidence.
Comparison between the new cohort (May-2007/Dec-2014) eICA-assessed before age 4 with wider but not systematic use of hydroxyurea and the earlier cohort (1988/Apr-2007) never eICAassessed until the 2008 update, revealed a significant reduction of SCI-risk (Log-Rank, P=.009) associated with eICA-assessment but not with wider use of hydroxyurea. eICA-TAMMVs≥160cm/s, even with no eICA-stenosis, were risk-factors for SCI, suggesting that all SCA-children with eICATAMMV≥ 160cm/s should be placed on chronic-transfusion. Hydroxyurea initiation at an early age was associated with lower intracranial-arteriopathy incidence, but not with lower eICA-arteriopathy and SCI-incidence. In the overall cohort (1988-2014), including 332 SCA-children, all assessed/managed for eICA-arteriopathy after 2011, the cumulative-SCI-incidence by age 14 was 25.0% (95%CI:19.0%-31.0%). SCI-risk was associated with being older at first-neck-MRA and having high MCV on hydroxyurea.
While the impact of hydroxyurea on SCI-incidence remains unclear, making controlled trials necessary, eICA-arteriopathy management by intensive therapy is effective at improving SCIprevention.
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| format | Article |
| id | doaj-art-ff196645764e42e9b675c1c3f0a93772 |
| institution | Kabale University |
| issn | 0390-6078 1592-8721 |
| language | English |
| publishDate | 2025-06-01 |
| publisher | Ferrata Storti Foundation |
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| spelling | doaj-art-ff196645764e42e9b675c1c3f0a937722025-08-20T03:28:01ZengFerrata Storti FoundationHaematologica0390-60781592-87212025-06-01999110.3324/haematol.2025.287720Early detection and management of extracranial arteriopathy reduces the incidence of silent cerebral infarcts in sickle cell anemia: a long-term prospective cohort studyFrancoise Bernaudin0Cecile Arnaud1Annie Kamdem2Jenny Youn3Manuela Vasile4Isabelle Hau5Fouad Madhi6Aline Malterre7Celine Delestrain8Ralph Epaud9Camille Jung10Suzanne Verlhac11Referral Center for Sickle Cell Disease, Department of Pediatrics, Centre Hospitalier Intercommunal, Creteil (CHIC Hospital), Creteil, University Paris XII, France; Clinical Research Department, CHIC Hospital, Universite Paris XIIReferral Center for Sickle Cell Disease, Department of Pediatrics, Centre Hospitalier Intercommunal, Creteil (CHIC Hospital), Creteil, University Paris XIIReferral Center for Sickle Cell Disease, Department of Pediatrics, Centre Hospitalier Intercommunal, Creteil (CHIC Hospital), Creteil, University Paris XIIReferral Center for Sickle Cell Disease, Department of Pediatrics, Centre Hospitalier Intercommunal, Creteil (CHIC Hospital), Creteil, University Paris XIIMedical Imaging Department, Referral Center for Sickle Cell Disease, CHIC Hospital, CreteilDepartment of Pediatrics, CHIC Hospital Creteil, Universite Paris XIIDepartment of Pediatrics, CHIC Hospital Creteil, Universite Paris XIIDepartment of Pediatrics, CHIC Hospital Creteil, Universite Paris XIIDepartment of Pediatrics, CHIC Hospital Creteil, Universite Paris XIIDepartment of Pediatrics, CHIC Hospital Creteil, Universite Paris XIIClinical Research Department, CHIC Hospital, Universite Paris XIIMedical Imaging Department, Referral Center for Sickle Cell Disease, CHIC Hospital, Creteil, France; Medical Imaging Department, Hopital Robert Debre, Paris Previous reports about the Creteil newborn-cohort (1988/Apr-2007) showed that the risk of silent cerebral infarcts (SCI) remained high (37.1%) by age 14 in children with sickle cell anemia (SCA) and intracranial time-averaged mean maximum velocity (TAMMV)≥200cm/s despite chronictransfusion. Systematic assessment of extracranial internal carotid artery (eICA) since June-2011 revealed that SCI-risk is associated with chronic or acute anemia and eICA-stenosis. Based on these results, SCA-children with eICA-TAMMV≥200cm/s or eICA-stenosis were placed on chronictransfusion and considered for allogeneic stem-cell transplantation (alloSCT). SCA-children with 160-199cm/s eICA-TAMMV were maintained on hydroxyurea. We hypothesized that detection/management of eICA-arteriopathy and wider use of hydroxyurea could reduce SCI-incidence. Comparison between the new cohort (May-2007/Dec-2014) eICA-assessed before age 4 with wider but not systematic use of hydroxyurea and the earlier cohort (1988/Apr-2007) never eICAassessed until the 2008 update, revealed a significant reduction of SCI-risk (Log-Rank, P=.009) associated with eICA-assessment but not with wider use of hydroxyurea. eICA-TAMMVs≥160cm/s, even with no eICA-stenosis, were risk-factors for SCI, suggesting that all SCA-children with eICATAMMV≥ 160cm/s should be placed on chronic-transfusion. Hydroxyurea initiation at an early age was associated with lower intracranial-arteriopathy incidence, but not with lower eICA-arteriopathy and SCI-incidence. In the overall cohort (1988-2014), including 332 SCA-children, all assessed/managed for eICA-arteriopathy after 2011, the cumulative-SCI-incidence by age 14 was 25.0% (95%CI:19.0%-31.0%). SCI-risk was associated with being older at first-neck-MRA and having high MCV on hydroxyurea. While the impact of hydroxyurea on SCI-incidence remains unclear, making controlled trials necessary, eICA-arteriopathy management by intensive therapy is effective at improving SCIprevention. https://haematologica.org/article/view/12140 |
| spellingShingle | Francoise Bernaudin Cecile Arnaud Annie Kamdem Jenny Youn Manuela Vasile Isabelle Hau Fouad Madhi Aline Malterre Celine Delestrain Ralph Epaud Camille Jung Suzanne Verlhac Early detection and management of extracranial arteriopathy reduces the incidence of silent cerebral infarcts in sickle cell anemia: a long-term prospective cohort study Haematologica |
| title | Early detection and management of extracranial arteriopathy reduces the incidence of silent cerebral infarcts in sickle cell anemia: a long-term prospective cohort study |
| title_full | Early detection and management of extracranial arteriopathy reduces the incidence of silent cerebral infarcts in sickle cell anemia: a long-term prospective cohort study |
| title_fullStr | Early detection and management of extracranial arteriopathy reduces the incidence of silent cerebral infarcts in sickle cell anemia: a long-term prospective cohort study |
| title_full_unstemmed | Early detection and management of extracranial arteriopathy reduces the incidence of silent cerebral infarcts in sickle cell anemia: a long-term prospective cohort study |
| title_short | Early detection and management of extracranial arteriopathy reduces the incidence of silent cerebral infarcts in sickle cell anemia: a long-term prospective cohort study |
| title_sort | early detection and management of extracranial arteriopathy reduces the incidence of silent cerebral infarcts in sickle cell anemia a long term prospective cohort study |
| url | https://haematologica.org/article/view/12140 |
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