MRI评估儿童异基因造血干细胞移植导致后部可逆性脑病综合征

目的分析后部可逆性脑病综合征（reversible posterior leukoencephalopathy syndrome, RPLS）患者的临床特征及影像学表现，以提高对该病的认识。方法回顾性分析2012年4月至2013年4月在我院接受异基因造血干细胞移植治疗后发生RPLS的10例患者的临床资料和影像学特征，总结其临床及影像学特征。结果8例患者有头痛、呕吐或高血压等前驱症状。9例患者出现抽搐发作，其中8例出现不同程度的视物模糊，1例抽搐后双眼失明1周。另1例仅表现为双眼失明。所有病例均做头颅MRI检查。MRI上都有具有特征性的后部皮层下脑白质内的融合性异常信号，在常规自旋回波T1WI及T2WI上表现为不确定的低信号及高信号，在FLAIR序列结合DWI序列上显示明显高信号，病灶边缘模糊不清，增强扫描未见明显异常强化。治疗后，所有患者在2周内症状缓解，得到MRI影像学证实，无永久后遗症。结论RPLS在儿童异基因造血干细胞移植治疗中并不罕见；头颅MRI检查和特征性临床表现能及时诊断、及时干预，复查MRI对判断治疗效果有较大帮助。

The feature of reversible posterior leukoencephalopathy syndrome after hematopoietic stem cell transplantation in children

Objective To analyze the clinic data and outcome of reversible posterior leukoencephalopathy syndrome （RPLS） after hematopoietic stem cell transplantation （HSCT） in children for further understanding this disease. Methods We retrospectively reviewed outcomes of 10 cases with PRLS after allogeneic-HSCT at our hospital between April of 2012 and April of 2013. Results 8 cases had prodromes with headache, vomiting or hypertension. 9 cases had seizures which 8 cases presented visual disturbances late and 1 cases blind for one week, 1 case just showed blindness for 2 days. All 10 cases presented with specific high signal intensities at the cortex and subcortical white matter of the parieto-occipital area, The margins were blur. There was no enhancement after contrast. Conclusion RPLS is not rare in children after allogeneic HSCT. The special clinic features and brain magnetic resonance imaging （MRI） can make a defmite diagnosis. Prompt interventions are helpful on these patients to improve their outcome.