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1例异基因骨髓移植后患有骨髓增生异常综合征的儿童发生后部可逆性脑病综合征。

A case of posterior reversible encephalopathy syndrome in a child with myelodysplastic syndrome following allogenic bone marrow transplantation.

作者信息

Heo Seonhee, Cho Hye Jung, Jeon In-sang

机构信息

Department of Pediatrics, Gil Medical Center, School of Medicine, Gachon University of Medicine and Science, Incheon, Korea.

出版信息

Pediatr Hematol Oncol. 2010 Feb;27(1):59-64. doi: 10.3109/08880010903420661.

DOI:10.3109/08880010903420661
PMID:20121556
Abstract

Posterior reversible encephalopathy syndrome (PRES) is an uncommon but distinctive clinical-radiologic entity characterized by headache, seizures, visual disturbance, and altered mental function associated with reversible white matter edema affecting the posterior parietal and occipital lobes of the brain. Although PRES is caused by a variety of conditions, acute elevation of blood pressure, fluid retention, and treatment with immunosuppressive drugs and/or anti-neoplastic agents are the main causes. A few cases of PRES associated with hematopoietic stem cell transplantation (HSCT) in children have been reported. Early recognition of PRES and appropriate management are needed to reduce the risk of permanent neurologic disability. The authors report a case of PRES in a girl who received an HLA-identical sibling bone marrow transplantation for myelodysplastic syndrome to emphasize the importance of early recognition and institution of appropriate management of PRES during HSCT.

摘要

后部可逆性脑病综合征(PRES)是一种罕见但独特的临床-放射学实体,其特征为头痛、癫痫发作、视觉障碍以及与影响大脑顶叶和枕叶的可逆性白质水肿相关的精神功能改变。尽管PRES由多种情况引起,但血压急性升高、液体潴留以及使用免疫抑制药物和/或抗肿瘤药物治疗是主要原因。已有少数儿童造血干细胞移植(HSCT)相关的PRES病例报道。需要早期识别PRES并进行适当管理,以降低永久性神经残疾的风险。作者报告了一例接受 HLA 相同同胞骨髓移植治疗骨髓增生异常综合征的女孩发生PRES的病例,以强调在HSCT期间早期识别和对PRES进行适当管理的重要性。

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