Clinical Analysis Of 19 Children With Hematology-related Posterior Reversible Encephalopathy Syndrome

Objective: To increase knowledge of posterior reversible encephalopathy syndrome(PRES)hematological disorders in children and reduce the occurrence of misdiagnosis,missed diagnosis and irreversible damage.Methods: By collecting the clinical data of 19 PRES children diagnosed and treated in our hematology ward from January 2015 to January 2019,the clinical manifestations,imaging examinations,laboratory examinations,treatment and prognosis were retrospectively analyzed.Results:1.A total of 19 cases were collected in this study,including 10 males and 9females with an average age of onset(8.2±2.8)years.Acute lymphoblastic leukemia,acute myelocytic leukemia,ALK-positive anaplastic large cell lymphoma,idiopathic thrombocytopenic purpura in 1 case each,14 cases after thalassemia hematopoietic stem cell transplantation and 1 case after hemophagocytic syndrome hematopoietic stem cell transplantation.2.The clinical manifestations were convulsions in 19 cases,recurrent convulsions in 4 cases,altered mental state in 14 cases,headache in 9 cases,vomiting in 7 cases,aconuresis or encopresis in 5 cases,dizziness in 4 cases,visual disturbances in 3 cases,fatigue in 2 cases,blood pressure increased significantly in 15 cases,normal or mild in 4 cases.3.There was no hypoglycemia in 19 cases,but hypocalcemia in 5 case,hyponatremia in 6 cases and hypomagnesemia in 9 cases.The cerebrospinal fluid(CSF)examination was performed in 11 cases.The CSF pressure increased in 2 cases,protein increased slightly in 2 cases and chlorine decreased slightly in4 cases.No abnormalities in cerebrospinal fluid routine,biochemical and etiological examination.The electroencephalogram(EEG)was performed in 9cases,8 cases were abnormal and 1 case was normal.4.The top five lesions of PRES were parietal lobe(100%),occipital lobe(84%),frontal lobe(58%),temporal lobe(32%),cerebellum(21%).The lesions showed iso-signal,low-signal or slightly long-signal on T1-weighted imaging,long-signal or high-signal on T2-weighted imaging,and slightly high-signal or high-signal on FLAIR.5.After symptomatic treatment in 19 cases,except for one case who gave up treatment due to severe intestinal GVHD,the clinical symptoms of the remaining 18 cases disappeared,11 cases were reviewed for skull MRI,6 cases showed less lesions,and 5 cases showed disappeared.Conclusion:1.There is no gender difference in the incidence of PRES.Immunosuppressive/cytotoxic drugs and hypertension may be risk factors for PRES in children with hematological diseases related.2.Compared with children receiving chemotherapy,children receiving hematopoietic stem cell transplantation may be more prone to PRES,while children receiving hematopoietic stem cell transplantation from thalassemia may be more prone to PRES than those receiving hematopoietic stem cell transplantation from other hematological/tumorous diseases.3.Convulsion and mental state change are the most common clinical symptoms of PRES in children.4.The main treatment of PRES is symptomatic treatment.The most important thing is to control blood pressure.5.Laboratory examination is non-specific.MRI was the first choice for diagnosis of PRES,and the lesions often involve the parietal and occipital lobes.6.Prompt diagnosis and treatment,PRES has a good prognosis.