Li Zuo-Feng, Xiong Hao, Chen Zhi, Yang Li, Sun Ming, Lu Wen-Jie, Qi Shan-Shan, Tao Fang, Luo Lin-Lin, Jiao Yu-Qing
Medical College of Wuhan University of Science and Technology, Wuhan 430065, Hubei Province, China.
Department of Hematology, Wuhan Children's Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430016, Hubei Province, China.
Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2024 Oct;32(5):1560-1565. doi: 10.19746/j.cnki.issn.1009-2137.2024.05.040.
To summarize the clinical features of reversible posterior encephalopathy syndrome (PRES) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) in children.
The clinical data of six children who developed PRES after undergoing allo-HSCT in the Department of Hematology of Wuhan Children's Hospital from June 2016 to December 2022 were retrospectively analyzed, and their clinical characteristics, imaging examination, laboratory examination, and treatment regression were summarized.
Among 281 children underwent allo-HSCT, 6 cases (2.14%) developed PRES, with a median age of 5.1(1.5-9.7) years old. 4 cases underwent related haploidentical donor transplantation, and 2 cases underwent sibling allografting and unrelated donor allografting donor transplantation, respectively. All six children had an acute onset of illness, with clinical manifestations of nausea and vomiting, seizures, psychiatric disorders, visual disturbances. The five cases elevated blood pressure. All children with PRES were treated with oral immunosuppressive drugs during seizures, and 3 cases were combined with different degrees of graft-versus-host disease. Most of the children showed effective improvement in clinical symptoms and imaging after adjusting/discontinuing suspected medications (cyclosporine, etc.) and symptomatic supportive treatments (oral antihypertensive, diazepam for antispasmodic, mannitol to lower cranial blood pressure), and one of them relapsed more than 8 months after the first seizure.
PRES is rare after hematopoietic stem cell transplantation in children, and its onset may be related to hypertension, cytotoxic drugs, graft-versus-host disease, etc. Most of them can be recovered after active treatment, but not completely reversible, and the prognosis of those who combined with TMA is poor.
总结儿童异基因造血干细胞移植（allo-HSCT）后可逆性后部脑病综合征（PRES）的临床特征。
回顾性分析2016年6月至2022年12月在武汉市儿童医院血液科接受allo-HSCT后发生PRES的6例儿童的临床资料，总结其临床特点、影像学检查、实验室检查及治疗转归。
281例接受allo-HSCT的儿童中，6例（2.14%）发生PRES，中位年龄5.1（1.5 - 9.7）岁。4例接受相关单倍体相合供者移植，2例分别接受同胞全相合移植及非血缘供者全相合移植。6例患儿均起病急，临床表现为恶心、呕吐、惊厥、精神障碍、视觉障碍。5例血压升高。所有PRES患儿在惊厥发作时均给予口服免疫抑制剂治疗，3例合并不同程度的移植物抗宿主病。多数患儿在调整/停用可疑药物（环孢素等）及对症支持治疗（口服降压药、地西泮解痉、甘露醇降颅压）后临床症状及影像学表现有效改善，其中1例在首次惊厥发作8个月后复发。
儿童造血干细胞移植后PRES少见，其发病可能与高血压、细胞毒性药物、移植物抗宿主病等有关。多数经积极治疗可恢复，但并非完全可逆，合并血栓性微血管病者预后差。
