Picchi Eliseo, Di Giuliano Francesca, Marziali Simone, Minosse Silvia, Ferrazzoli Valentina, Da Ros Valerio, Gaziev Javid, Pistolese Chiara Adriana, Floris Roberto, Garaci Francesco
Diagnostic Imaging and Neuroradiology, Department of Biomedicine and Prevention, University of Rome Tor Vergata, Via Montpellier 1, 00133 Rome, Italy.
International Center for Transplantation in Thalassemia and Sickle Cell Anemia. Mediterranean Institute ofHematology, University Hospital of Rome "Tor Vergata", Viale Oxford 81, 00133 Rome, Italy.
Eur J Radiol Open. 2019 Apr 16;6:144-151. doi: 10.1016/j.ejro.2019.03.001. eCollection 2019.
To evaluate, by Magnetic Resonance Imaging, if there is a typical pattern or severity of PRES in transplanted children for hemoglobinopathy. Secondary point was to investigate the pattern and severity of PRES in children with thalassemia-THAL and sickle-cell disease-SCD after autologous hematopoietic stem cell transplantation (aHSCT). Finally, we evaluate the presence of atypical PRES presentation and the involved area of central nervous system. Two neuroradiologists analyzed retrospectively MRI of 21 transplanted children for THAL or SCD treated with CI, with neurological symptoms and signs of PRES. The Bartynski and Boardman classification has been used for PRES pattern while McKinney scale for PRES severity. Fisher Exact Probability test or Chi-square test were used to compare the categorical data. In the 21 transplanted children the PRES severity was typically mild (85.7%) without preferring radiological pattern at MRI. The analysis didn't show significant association between PRES pattern or PRES severity and previous hemoglobinopathy (THAL or SCD). No atypical PRES presentation has been found. PRES severity in transplanted children for hemoglobinopathy is typically mild. Notwithstanding children affected by SCD have a damage on the capillary endothelium, after aHSCT our data didn't show a different PRES severity and pattern than THAL children.
通过磁共振成像评估患有血红蛋白病的移植儿童是否存在典型的后部可逆性脑病综合征(PRES)模式或严重程度。次要目的是研究地中海贫血(THAL)和镰状细胞病(SCD)儿童在自体造血干细胞移植(aHSCT)后PRES的模式和严重程度。最后,我们评估非典型PRES表现的存在情况以及中枢神经系统受累区域。两名神经放射科医生回顾性分析了21例接受CI治疗、有PRES神经症状和体征的THAL或SCD移植儿童的MRI。PRES模式采用Bartynski和Boardman分类,PRES严重程度采用McKinney量表。使用Fisher精确概率检验或卡方检验比较分类数据。在21例移植儿童中,PRES严重程度通常为轻度(85.7%),MRI上无明显的放射学模式偏好。分析未显示PRES模式或严重程度与既往血红蛋白病(THAL或SCD)之间存在显著关联。未发现非典型PRES表现。患有血红蛋白病的移植儿童的PRES严重程度通常为轻度。尽管患有SCD的儿童毛细血管内皮有损伤,但在aHSCT后,我们的数据显示其PRES严重程度和模式与THAL儿童并无不同。