Posterior Reversible Encephalopathy Syndrome After Allogeneic Hematopoietic Stem Cell Transplantation

Objective: We investigated the incidence,clinical characteristics,risk factors and prognosis of posterior reversible encephalopathy syndrome(PRES)after allogeneic hematopoietic stem cell transplantation(allo-HSCT),in order to provide clinical experience for the timely diagnosis and treatment of PRES.Methods: We retrospectively analyzed the clinical data of 815 patients with hematopathy receiving allo-HSCT in our hospital from September 2006 to October 2018.Results: In our 815 patients,a total of 44 patients developed PRES after allo-HSCT,with an incidence of 5.6%.The median time of PRES onset was41.5days(range,4 to 367 days),17 patients(38.6%)developed it within 30 days and 39 patients(88.6%)developed it within 100 days after transplantation.The clinical manifestations of PRES in our 44 patients mainly included hypertension(90.9%),seizures(81.8%),altered mental status(52.3%)and headache(34.1%).Univariate analysis showed that age,hypertension,patients with thalassemia major(TM),type of transplantation and acute graft-versus-host disease(aGVHD)were significantly correlated with the development of PRES.The multivariate analysis showed that hypertension(HR=10.440,95%CI 3.723~29.274,P<0.001),patients with TM(HR=2.811,95%CI 1.472~5.369,P=0.002),haploid transplantation(HR=2.986,95%CI 1.217~7.325,P=0.017)and aGVHD(HR=2.345,95%CI 1.258~4.373,P=0.007)were independent risk factors for PRES.After the diagnosis of PRES,44 patients immediately withdrew CNIs and gave positive symptomatic treatment including controlling hypertension,sedative treatment,and treating seizures.Finally,all patients experienced full neurological recovery except for 2 patients who died of severe pneumonia had not received complete recovery at the time of death.Ultimately,all patients in our study were followed up to February 2019,and a total of 593 patients survived,with a survival rate of 72.8%.The median follow-up time after transplantation was 29 months(range,1 to 149 months).In the 44 patients with PRES,18 patients(40.9%)died.Univariate analysis showed that age ? 40,malignant hematopathy,stage of disease progression,type of transplantation,grades ? to IV aGVHD and PRES were significantly correlated with the survival of patients with hematopathy after allo-HSCT.The multivariate analysis showed that age? 40,malignant hematopathy,stage of disease progression,grades ? to IV aGVHD and PRES were independent risk factors for survival.The overall survival between the patients who had PRES and the patients who did not have PRES were significantly different(58.0% versus70.3%,P=0.009).The overall survival between the patients with TM who had PRES and those without PRES were significantly different(76.3% versus 94.2%,P<0.001).Conclusion: 1.The incidence of PRES in patients with hematopathy after allo-HSCT is 5.6%,which mostly occurred within 100 days after transplantation.Hypertension,seizures,disturbance consciousness and headache were the main clinical manifestations of PRES.2.Hypertension,patients with TM,haploid transplantation,and aGVHD were independent risk factors of PRES.3.PRES is one of the independent risk factors of survival for patients with hematopathy receiving allo-HSCT.The overall survival of patients with PRES is significantly lower than the patients without PRES.Early diagnosis and timely treatment is the key to improve the poor prognosis of PRES.