Objective: To summarize the clinical features of reversible posterior encephalopathy syndrome (PRES) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) in children.
Methods: 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.
Results: 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.
Conclusion: 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.
题目: 儿童异基因造血干细胞移植后可逆性后部脑病综合征的临床分析.
目的: 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（posterior reversible encephalopathy syndrome，PRES）的临床特征。.
方法: 回顾性分析2016年6月-2022年12月在武汉儿童医院血液内科接受allo-HSCT后发生PRES的6例患儿临床资料，对其临床特性、影像学检查、实验室检查及治疗转归等进行总结。.
结果: 共有281例患儿接受allo-HSCT，其中6例（2.14%）发生PRES，中位年龄5.1（1.5-9.7）岁。4例接受亲缘单倍体供者移植，2例分别接受同胞全相合及无关供者全相合供者移植。6例患儿均为急性起病，临床表现以恶心呕吐、癫痫发作、精神障碍、视力障碍为主，5例均伴血压升高。所有PRES患儿发作期间均接受口服免疫抑制剂药物治疗，3例次合并不同程度移植物抗宿主病，经调整/停用可疑药物（环孢素等）及对症支持处理（口服降压药、地西泮静推止痉、甘露醇降颅压）后大部分患儿临床症状和影像学均有效改善，其中1例于首次发作8月余后复发。.
结论: 儿童造血干细胞移植后发生PRES少见，其发病可能与高血压、细胞毒性药物、移植物抗宿主病等因素相关，经积极治疗大部分可恢复，但不完全可逆，合并TMA者预后差。.
Keywords: reversible posterior encephalopathy syndrome; allogeneic hematopoietic stem cell transplantation; children; thrombotic microangiopathy.