急性淋巴细胞白血病患儿诱导缓解期合并可逆性后部白质脑病临床特征

摘要

Objective To investigate the etiology,clinical manifestations,imaging characteristics and treatment of reversible posterior leukoencephalopathy syndrome in children with acute lymphocytic leukaemia after remission induction chemotherapy.Methods Analysize the clinico-radiological features、central nervous system symptoms and associated symptoms、treatment and prognosis of reversible posterior leukoencephalopathy syndrome in children with acute lymphocytic leukaemia receiving induction chemotherapy in hematology center of Beijing children′s hospita from June 201 1 to March 2012.Results Eight children (3 males and 5 females)with a mean age of 5 years were identified.Presenting symptoms included seizures (8/8 ),disturbance of consciousness (3/8 ),and visual disturbance (2/8 ).High blood pressure,agranulocytosis,and coagulation disorders,electrolyte disturbance were existed,and they all used granulocyte colony-stimulating factor.All children had typical radiological features of reversible posterior leukoencephalopathy syndrome.Six cases of children with head magnetic resonance imaging results suggest that vasogenic edema,the prognosis is good.Two cases of cytotoxic edema,in one case of recurrence,the prognosis is bad.Conclusion reversible posterior leukoencephalopathy syndrome is an underappreciated complication in cancer children receiving remission induction chemotherapy.Head magnetic resonance imaging is an important means of diagnosis and assessment of reversible posterior leukoencephalopathy syndrome prognosis. During chemotherapy require close monitoring of blood pressure,electrolyte,blood coagulation,actively is needed.%目的：探讨急性淋巴细胞白血病（acute lymphoblastic leukemiu，ALL）患儿，在诱导缓解化疗期间发生可逆性后部白质性脑病综合征（reversible posterior leukoencephalopathy sysdrome，RPLS）的病因、临床表现、影像学特征及治疗。方法2011年6月至2012年3月，北京儿童医院血液病中心诱导缓解化疗期间出现RPLS的8例患儿。回顾性分析RPLS发病时间、临床表现、影像学检查特点、治疗及预后。结果8例患儿发生RPLS中位时间为诱导缓解化疗第28（23～34）d，以癫痫为主要表现，2例视觉障碍，3例意识障碍；均有粒细胞缺乏、电解质紊乱及凝血功能障碍；应用过粒细胞集落刺激因子；8例患儿均存在典型头部核磁共振成像（MRI ）特征，6例患儿出现血管源性水肿，预后良好，2例患儿出现细胞毒性水肿，其中1例复发，预后不良。结论 RPLS可发生在ALL患儿诱导缓解化疗期间，有特征性临床和影像学检查表现，头部MRI检查是诊断及评估RPLS预后的重要手段。其发病机制暂不明确。在化疗期间需要密切监测血压、血常规、电解质及凝血功能，积极处理。