Kapoor Rajan, Simalti Ashish, Kumar Rajiv, Yanamandra Uday, Das Satyaranjan, Singh Jasjit, Nair Velu
Departments of Medicine and Clinical Hematology.
Department of Pediatrics, Base Hospital, Delhi Cantt.
J Pediatr Hematol Oncol. 2018 Aug;40(6):433-437. doi: 10.1097/MPH.0000000000001190.
Posterior reversible encephalopathy syndrome (PRES) has diverse etiologies and is closely linked to hematopoietic stem cell transplant (HSCT). Headache and seizures are the most common clinical presentations. Although near total recovery is seen in the majority of patients with appropriate management, the implications of its occurrence in the setting of an HSCT is much more than the residual neurological deficits. Graft rejection and occurrence of graft versus host disease has been reported. We analyzed retrospectively our data of 35 pediatric HSCT recipients over the last 2 years at our center. In total, 17% (n=6) patients developed PRES. Headache and seizures were the most common clinical presentations. All patients were on calcineurin inhibitors at the onset of symptoms. The median time after HSCT to the onset of PRES was 21 days. In total, 34% (n=2) patients developed residual neurological deficit. One patient died of acute graft versus host disease at a later date, and 50% (n=3) patients had graft rejection and return to transfusion dependence. The implications of PRES on HSCT outcomes are grave, and better immunosuppression transition protocols need to be developed.
后部可逆性脑病综合征（PRES）病因多样，与造血干细胞移植（HSCT）密切相关。头痛和癫痫是最常见的临床表现。尽管大多数患者经过适当治疗后几乎可完全康复，但在HSCT背景下其发生的影响远不止残留的神经功能缺损。已有报道发生移植物排斥和移植物抗宿主病。我们回顾性分析了本中心过去2年35例儿科HSCT受者的数据。共有17%（n = 6）的患者发生了PRES。头痛和癫痫是最常见的临床表现。所有患者在症状出现时均使用钙调神经磷酸酶抑制剂。HSCT至PRES发病的中位时间为21天。共有34%（n = 2）的患者出现残留神经功能缺损。1例患者后来死于急性移植物抗宿主病，50%（n = 3）的患者发生移植物排斥并恢复输血依赖。PRES对HSCT结局的影响严重，需要制定更好的免疫抑制转换方案。
