儿童急性淋巴细胞白血病合并可逆性后部脑病综合征的临床特征分析

Clinical characteristics of posterior reversible encephalopathy syndrome in pediatric acute lymphoblastic leukemia

  • 摘要: 目的 探讨儿童急性淋巴细胞白血病(acute lymphoblastic leukemia,ALL)化疗期间合并可逆性后部脑病综合征(posterior reversible encephalopathy syndrome,PRES)的临床特征,提高对本病的认识。方法 回顾性分析2008年11月-2019年1月在解放军总医院第一医学中心小儿内科就诊,明确诊断为ALL且合并PRES的14例患儿临床资料。结果 14例患儿中男性5例,女性9例,中位年龄6(2~13)岁。12例患儿在诱导缓解治疗(VDLP方案)期间发生PRES,发病中位时间为化疗第16(4~28)天。除PRES典型症状外,临床上可见腹痛、自动症等特征性症状。损伤在MRI影像学上表现为后头部双侧对称长T1长T2信号、DWI呈高信号灶。脑电图可见背景慢波、痫样放电等特征性改变。症状持续中位时间为15(4~26) d,经对症治疗和调整化疗方案后,12例患儿症状完全缓解,1例遗留皮质盲,1例在症状未缓解前死亡,1例症状缓解后在序贯化疗中再次发生PRES。根据PRES预警评分量表,将患儿分为脑电图高危组(8例)和脑电图低危组(6例)。2例未缓解病例均在高危组,患儿症状持续时间与脑电图危险度分组呈正相关,相关系数为0.864(P=0.001)。结论 ALL患儿化疗期间合并PRES主要发生于诱导缓解化疗期间,患儿较特殊的临床表现为腹痛和自动症。床旁脑电图检查能协助临床早期诊断,并对判断患儿预后具有一定临床意义。

     

    Abstract: Objective To explore the clinical characteristics of posterior reversible encephalopathy syndrome (PRES) during chemotherapy in children with acute lymphocytic leukemia (ALL),and improve the understanding of the disease. Methods A retrospective analysis was performed on clinical data about 14 children with a definite diagnosis of ALL and PRES in the First Medical Center of Chinese PLA General Hospital from November 2008 to January 2019. Results Of the 14 cases,there were 5 males and 9 females,with a median age of 6 (2-13) years.Twelve cases developed PRES during remission of induction chemotherapy,and the median time from chemotherapy to onset was 16 (4-28) days.Except for typical symptoms,the clinical manifestations of PRES were abdominal pain and autism.MRI showed that the lesions were symmetrically distributed in the parietal-occipital lobes and portion cortical,presenting as hyperintensity on T1WI,T2WI and DWI sequences.Typical EEG features were visible slow waves in the background,and epilepsy-like discharges were occasionally seen.After treatment,12 patients achieved complete remission,and the median duration from symptom onset to cure was 15 (4-26) day;one patient developed irreversible visual impairment,one patient died before achieving complete remission,and one patient relapsed during sequential chemotherapy.According to the early warning scoring of PRES,cases were divided into electroencephalogram (EEG) low-risk group (6 cases) and EEG high-risk group (8 cases).In the EEG high-risk group,the duration of symptoms was positively correlated with EEG score with a correlation coefficient of 0.864 (P=0.001). Conclusion PRES frequently occurs during remission of induction chemotherapy,and its clinical features are abdominal pain and autism.The EEG can assist early clinical diagnosis and has certain clinical significance for predicting the prognosis of children.

     

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