血液病/肿瘤患儿并发可逆性后部白质脑病综合征

Clinical characteristics analysis of children with reversible posterior leukoen-cephalopathy syndrome during the treatment of hematological tumor

目的:探讨血液病/肿瘤患儿并发可逆性后部白质脑病综合征(reversible posterior leukoen-cephalopathy syndrome,RPLS)惊厥前后的临床特点,加深对RPLS的理解。方法:回顾性分析2012年3月至2017年3月在北京大学第一医院儿科血液/肿瘤病房发生RPLS的5例患儿的临床资料,包括年龄、性别、基础疾病、有否肾受累、体重指数、是否有高血压家族史等基本临床资料,同时检测其发生惊厥前后的血红蛋白水平、血压及血清钠水平,并随访记录患儿预后,包括原发病情况及神经系统症状体征的恢复状况。结果:患儿均为学龄期或学龄前期女童,所患基础疾病4例为伴有肾受累或一侧肾切除的恶性肿瘤,1例为难治性自身免疫性溶血性贫血。所有患儿都存在轻、中度贫血。RPLS发生前一天应用环磷酰胺、长春新碱和放线菌素D化疗,或者应用环孢素A和糖皮质激素治疗。患儿发生RPLS惊厥时表现为晨起或午睡起突发的无热惊厥,之后即刻检查,发现与自身相比,其血压较发作前明显升高,同时血清钠降低,经用地西泮、呋噻米和氨氯地平等治疗后很快恢复。4例未遗留后遗症,1例遗留有情感及行为水平变幼稚,康复2年后逐渐恢复。复习文献中主要是报道血压的升高。结论:并发RPLS的血液病/肿瘤患儿可能多有肾受累和贫血,血压升高同时伴血清钠降低可能在RPLS发病中起一定作用,同时保持血压稳定和电解质平衡有可能减少RPLS的发生或减轻其严重程度。

Objective： To analyze the clinical characteristics of patients with hematological tumor or disease before and after reversible posterior leukoen-cephalopathy syndrome（RPLS）. Methods： Five patients were both from Peking University First Hospital Pediatric Hematology-oncology Department in the period from March 2012 to March 2017. The gender,age,BMI,underlying diseases,with or without renal damage,hypertension family history,clinical manifestations of convulsions,hemoglobin,and blood pressure,serum sodium levels before and after convulsion,and other data of the children with RPLS were retrospectively analyzed. In the meantime,we followed up the five patients for 6 months to 66 months,kept a watchful eye on their original condition and the recovery of symptoms and signs of the nervous system. The relevant literature was reviewed. Results： All of the subjects were females in school-age or preschool age. The underlying diseases were malignant tumor associated with renal involvement or on one side of nephrectomy in 4 of these subjects,while the other one was refractory autoimmune hemolytic anemia. All of the subjects suffered from mild or moderate anemia. The day before RPLS occurred they received chemotherapy made up with cyclophosphamide,vincristine,and actinomycin-D,or the therapy with cyclosporin A and glucocorticoid. The clinical manifestations were afebrile convulsion after getting up in the mooring or in the afternoon. We observed elevation of blood pressure and cutting down of serum sodium compared with themselves. All of the cases recovered soon after management with diazepam,furosemide and amlodipine besylate. Four of them had a good outcomes and did not remain any sequela,while only one girl became childish in emotion and behavior,and then returned gradually to normal two years later. However,by long-term follow-up,the elevation of blood pressure was mainly reviewed in literature. Conclusion： The patients attacked by RPLS,with hematology or oncology cases,could have the underlying disease of renal damage and anemia. Blood pressure elevation and serum sodium falling down at the same time may play an important role during the occurrence of RPLS. Remaining stable of blood pressure and electrolyte level together will possibly reduce or mitigate RPLS.