处理镰状细胞病的脑血管并发症:目前的观点。

IF 2.1 Q3 HEMATOLOGY
Jennifer Light, Maria Boucher, Jacquelyn Baskin-Miller, Mike Winstead
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引用次数: 1

摘要

保护镰状细胞病(SCD)患者脑功能的重要性怎么强调都不为过。SCD伴有多种脑血管并发症,威胁神经认知功能和生命。如果没有筛查和预防管理,11%的儿童和24%的成人SCD患者会发生缺血性或出血性中风。采用经颅多普勒超声(TCD)筛查SCD患儿脑卒中是一种行之有效的方法。TCD速度超过200厘米/秒会显著增加中风的风险,这可以通过慢性红细胞(RBC)输血来预防。输血也是急性脑卒中管理和二级脑卒中预防的基石。慢性输血需要对铁超载等并发症进行长期管理。羟基脲可在特定患者组或慢性输注不可行的人群中替代慢性输注用于初级脑卒中预防。无症状性脑梗死(SCI)甚至比中风更常见,影响39%的儿童和50%以上的SCD成人;脊髓损伤的治疗是个体化的,包括仔细的神经认知评估。造血干细胞移植可以预防脑血管并发症,尽管有短期和长期的风险。较新的疾病调节剂,如voxelotor和crizanlizumab,以及基因疗法,可能治疗脑血管并发症,但这些方法尚处于研究阶段。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Managing the Cerebrovascular Complications of Sickle Cell Disease: Current Perspectives.

Managing the Cerebrovascular Complications of Sickle Cell Disease: Current Perspectives.

Managing the Cerebrovascular Complications of Sickle Cell Disease: Current Perspectives.

Managing the Cerebrovascular Complications of Sickle Cell Disease: Current Perspectives.

The importance of protecting brain function for people with sickle cell disease (SCD) cannot be overstated. SCD is associated with multiple cerebrovascular complications that threaten neurocognitive function and life. Without screening and preventive management, 11% of children at 24% of adults with SCD have ischemic or hemorrhagic strokes. Stroke screening in children with SCD is well-established using transcranial Doppler ultrasound (TCD). TCD velocities above 200 cm/s significantly increase the risk of stroke, which can be prevented using chronic red blood cell (RBC) transfusion. RBC transfusion is also the cornerstone of acute stroke management and secondary stroke prevention. Chronic transfusion requires long-term management of complications like iron overload. Hydroxyurea can replace chronic transfusions for primary stroke prevention in a select group of patients or in populations where chronic transfusions are not feasible. Silent cerebral infarction (SCI) is even more common than stroke, affecting 39% of children and more than 50% of adults with SCD; management of SCI is individualized and includes careful neurocognitive evaluation. Hematopoietic stem cell transplant prevents cerebrovascular complications, despite the short- and long-term risks. Newer disease-modifying agents like voxelotor and crizanlizumab, as well as gene therapy, may treat cerebrovascular complications, but these approaches are investigational.

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来源期刊
CiteScore
3.50
自引率
0.00%
发文量
94
审稿时长
16 weeks
期刊介绍: The Journal of Blood Medicine is an international, peer-reviewed, open access, online journal publishing laboratory, experimental and clinical aspects of all topics pertaining to blood based medicine including but not limited to: Transfusion Medicine (blood components, stem cell transplantation, apheresis, gene based therapeutics), Blood collection, Donor issues, Transmittable diseases, and Blood banking logistics, Immunohematology, Artificial and alternative blood based therapeutics, Hematology including disorders/pathology related to leukocytes/immunology, red cells, platelets and hemostasis, Biotechnology/nanotechnology of blood related medicine, Legal aspects of blood medicine, Historical perspectives. Original research, short reports, reviews, case reports and commentaries are invited.
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