儿童血液肿瘤化疗期间合并可逆性后部脑病综合征5例临床分析并文献复习

Posterior reversible encephalopathy syndrome during chemotherapy in children with hematological or tumor diseases:clinical analysis of 5 cases and literature review

目的探讨儿童血液肿瘤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2013年11月—2020年11月在山东第一医科大学附属省立医院小儿血液肿瘤科就诊,明确诊断为儿童血液肿瘤疾病(包括白血病、淋巴瘤)且合并PRES的5例患儿的临床资料。结果5例患儿中男性3例,女性2例,年龄2岁5个月~12岁。患儿确诊后在我院规律化疗,无原发病复发,其中4例PRES发生于本次化疗的第8~28d,1例发生于维持治疗时,起病时5例患儿均伴有严重感染(其中1例为细菌感染,1例为真菌感染,3例为细菌及真菌合并感染),其中5例同时伴有电解质紊乱(5例低钠血症、4例低钾血症),4例伴有血压升高。临床特征主要表现为头痛、意识不清、癫痫发作(4例为全面性强直-阵挛性发作),伴呕吐、流涎、精神淡漠、尿失禁。起病后即刻行头颅MRI检查显示颞叶、顶叶、枕叶、小脑、丘脑等部位T2WI高信号,FLAIR高信号,MRI显示病灶与患儿临床特征、神经系统查体定位基本一致。经脱水降颅压、抗感染、补充电解质及其他对症支持治疗1~2周后,5例患儿的临床症状好转,后期化疗均按原方案、原剂量进行。2~3个月后复查MRI病灶完全或大部分消失,半年后5例患儿均未复发,4例未遗留神经系统后遗症,1例遗留下肢肌力减低。结论血液肿瘤疾病儿童在化疗期间合并PRES主要发生于化疗疗程后期,化疗后合并消化道感染、应用抗真菌药物及肿瘤负荷大的患儿更易发生PRES,发病时多合并有血压增高,颅脑MRI多有特征性改变,能协助临床早期诊断,并对患儿预后评估具有一定临床意义,控制血压及对症支持治疗后多预后良好。

Objective To investigate the clinical characteristics of posterior reversible encephalopathy syndrome(PRES)during chemotherapy in children with hematological or tumor diseases,so as to improve the understanding of this disease.Methods Clinical data of 5 children with pediatric hematological or tumor diseases(including leukemia,lymphoma)complicated with PRES in the Department of Pediatric Hematology,Provincial Hospital Affiliated to Shandong First Medical University from November 2013 to November 2020 were analyzed retrospectively.Results Among all 5 patients,there were 3 males and 2 females,aged from 2 years and 5 months to 12 years old.After diagnosis,the children received regular chemotherapy in our hospital without recurrence of primary disease.Among them,4 cases of PRES occurred on the 8th to 28th day of chemotherapy course,1 case occurred during maintenance treatment,and all 5 cases were accompanied by severe infection at the onset of disease(including 1 case of bacterial infection,1 case of fungal infection,and 3 cases of combined bacterial and fungal infection).Five patients were accompanied by electrolyte disturbance(5 with hyponatremia,4 with hypokalemia)and 4 had high blood pressure.The main clinical features were headache,unconsciousness,epileptic seizures(4 cases were generalized tonic-clonic seizures),accompanied by vomiting,salivation,apathy,and urinary incontinence.Immediate magnetic resonance imaging(MRI)examination after disease onset showed high T2WI signal and high FLAIR signal in the temporal lobe,parietal lobe,occipital lobe,cerebellum,thalamus and other parts.The lesions showed in MRI were basically consistent with the clinical features,the physical examination and positioning of the nervous system.After dehydration to reduce cranial pressure,anti-infection,electrolyte supplement and other symptomatic supportive treatments for 1-2 weeks,the clinical symptoms of the 5 children improved,and the late chemotherapy was carried out according to the original plan.After 2 to 3 months,the MRI lesions completely or mostly disappeared.Half a year later,5 cases had no recurrence,4 cases had no neurological sequelae,and 1 case had lower limb muscle weakness.Conclusions Childhood hematological or tumor diseases complicated with PRES during chemotherapy mainly occur at the late stage of chemotherapy course.Children with gastrointestinal infection,antifungal drugs and large tumor load after chemotherapy are more likely to develop PRES.At the onset,there are often increased blood pressure and characteristic changes in cranial MRI,which can assist in early clinical diagnosis and prognosis evaluation.The prognosis is good after the control of blood pressure and symptomatic supportive treatment.