Posterior Reversible Encephalopathy Syndrome after Allogeneic Stem Cell Transplantation in Pediatric Patients with Fanconi Anemia, a Prospective Study

IF 4.3 Q1 Medicine
Maryam Behfar , Mohammad Babaei , Amir Reza Radmard , Soheil Kooraki , Hamid Farajifard , Parisa Naji , Sahar Taebi , Amir Ali Hamidieh
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引用次数: 5

Abstract

Posterior reversible encephalopathy syndrome (PRES) is one of the most common neurologic complications following hematopoietic stem cell transplantation (HSCT). We aimed to evaluate the incidence, clinical, and imaging features of PRES in pediatric patients with Fanconi anemia (FA) following HSCT. This prospective study included all post-HSCT patients with underlying FA disease between 2014 and 2017. Brain computed tomography scan and magnetic resonance imaging (MRI) were performed in all individuals who developed neurologic symptoms. PRES was diagnosed based on clinic-radiological evidence. Follow-up MRI was performed in all patients with PRES within two months. Forty-one patients with FA (28 males; mean age, 8.19 ± 3.25 years) were enrolled. Out of 15 patients with acute neurologic symptoms, PRES was diagnosed in 9 individuals (21.95% of the total cohort). The occurrence of PRES was significantly higher in patients who had a donor with a 1-locus mismatch (P= .02). Donor relation, stem cell source, and graft-versus-host disease grade did not have any significant association with the development of PRES. MRI showed asymmetric vasogenic edema in 5 patients, an overt infarct in 1 patient, and foci of microhemorrhages in 3 patients, 1 of whom developed a hemorrhagic infarct. This patient died shortly, and persistent microhemorrhages were noted in the other 2 patients. Our findings demonstrate a greater risk of developing PRES after HSCT in patients with FA compared with those with other diseases (21.95% versus 1% to 10%), and in contrast to its term, it might be irreversible and has adverse effects on HSCT outcomes. The increased vascular and endothelial fragility in FA may contribute to the higher frequency of PRES in these individuals.

儿童范可尼贫血患者异体干细胞移植后后部可逆性脑病综合征的前瞻性研究
后可逆性脑病综合征(PRES)是造血干细胞移植(HSCT)后最常见的神经系统并发症之一。我们的目的是评估HSCT后儿童范可尼贫血(FA)患者PRES的发生率、临床和影像学特征。这项前瞻性研究纳入了2014年至2017年间所有患有潜在FA疾病的hsct后患者。对所有出现神经系统症状的个体进行脑计算机断层扫描和磁共振成像(MRI)。PRES是根据临床放射学证据诊断的。所有PRES患者均在2个月内随访MRI。FA 41例(男性28例;平均年龄(8.19±3.25岁)。在15例有急性神经系统症状的患者中,有9例(占总队列的21.95%)被诊断为PRES。1位点不匹配的供体患者的PRES发生率显著高于1位点不匹配的供体(P= 0.02)。供体关系、干细胞来源和移植物抗宿主病分级与PRES的发生无显著相关性。MRI显示5例患者血管源性水肿不对称,1例患者有明显梗死,3例患者有微出血灶,其中1例发生出血性梗死。该患者很快死亡,另外2例患者出现持续性微出血。我们的研究结果表明,与其他疾病患者相比,FA患者在HSCT后发生PRES的风险更高(21.95%比1% - 10%),并且与其期限相反,它可能是不可逆的,并且对HSCT结果有不利影响。FA患者血管和内皮易碎性的增加可能导致这些患者发生PRES的频率更高。
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来源期刊
CiteScore
6.60
自引率
0.00%
发文量
1061
审稿时长
3-6 weeks
期刊介绍: Biology of Blood and Marrow Transplantation publishes original research reports, reviews, editorials, commentaries, letters to the editor, and hypotheses and is the official publication of the American Society for Transplantation and Cellular Therapy. The journal focuses on current technology and knowledge in the interdisciplinary field of hematopoetic stem cell transplantation.
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