Clinical Analysis of Reversible Posterior Encephalopathy Syndrome after Allogeneic Hematopoietic Stem Cell Transplantation in Children
10.19746/j.cnki.issn1009-2137.2024.05.040
- VernacularTitle: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
- Author:
Zuo-Feng LI
1
;
Hao XIONG
;
Zhi CHEN
;
Li YANG
;
Ming SUN
;
Wen-Jie LU
;
Shan-Shan QI
;
Fang TAO
;
Lin-Lin LUO
;
Yu-Qing JIAO
Author Information
1. 武汉科技大学医学院,湖北武汉 430065
- Keywords:
reversible posterior encephalopathy syndrome;
allogeneic hematopoietic stem cell transplantation;
children;
thrombotic microangiopathy
- From:
Journal of Experimental Hematology
2024;32(5):1560-1565
- CountryChina
- Language:Chinese
-
Abstract:
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.
