Objective: To illustrate clinical features, treatment and prognosis of CNI induced PRES. Method: Clinical data, treatment and sequelae of patients who developed PRES in 171 HSCT patients were analyzed. Result: Five (2.9% ) patients with hematological malignancies were diagnosed of CNI induced PRES after HSCT. The median age was 2Q (16-28 ) years old. All 5 patients received matched unrelated or mismatched related HSCT (two received matched unrelated HSCT, among whom 3 received mismatched related HSCT). The common clinical manifestations were conscious disturbance and convulsion. Blood concentration of CNI were elevated in 3 patients. All patients undertook MRI which revealed patchy or multi-areas of increased signal intensity in white matter on T2WI. All 5 patients discontinued CNI and received supportive treatment One patient died of cerebral hemorrhage , 4 other patients recovered and without relapse. Conclusion: PRES is an uncommon complication of CNS related to CNI. If it is diagnosed and treated early, the prognosis of PRES is favorable.%目的:分析并探讨钙调神经磷酸酶抑制剂(CNI)相关的后部可逆性脑病综合征(PRES)的临床特点、处理方法和预后.方法:对171例并基因造血干细胞移植术患者中发生PRES的临床资料、治疗及转归等进行分析总结.结果:5例(2.9％)在进行造血干细胞移植后合并CNI相关的 PRES,年龄16 ～28岁,中位年龄20岁.5例均为非亲缘及亲缘非全相合移植,其中2例接受非血缘全相合移植,3例接受亲缘非全相合移植.5例的临床表现以意识障碍、抽搐为主,2例CNI浓度偏高,5例头颅MRd的T2加权像均表现为白质区多发或片状高信号病灶.出现PRES后,所有患者均停用CNI并予对症治疗.1例患者因脑出血死亡,4例患者病情缓解且无复发.结论:PRES是CNI少见的中枢神经系统并发症,及早发现,处理及时,预后较佳.
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