Posterior Reversible Encephalopathy Syndrome in Pediatric Hematopoietic Stem Cell Transplantation with Beta Major Thalassemia: The Association between the PRES Occurrence and Class of Beta Major Thalassemia

Abstract Allogeneic hematopoietic stem cell transplantation (HSCT) is the only definitive curative option for β-major thalassemia patients (β-MT). Posterior reversible encephalopathy syndrome (PRES) is a pervasive neurological complication which typically ensues HSCT. β-MT patients are prone to higher PRES incidence compared to other hematologic and non-hematologic disorders. Hence, these patients must be evaluated for PRES after HSCT. We included 148 (90 males and 58 females) β-MT pediatric patients who underwent HSCT between March 2015 and August 2022 in our Center. Patients were followed for 5 years. In tandem, based on our findings, the clinical imaging findings in 25 pediatric patients, which represent 16.9% of the cohort, corroborated the diagnosis of PRES. 14 of 112 patients (12%) with class I and II developed PRES however, 11 of 36 patients (30.5%) with β-MT-III indicated PRES manifestation. Our results indicated there was a significant correlation between class β-MT-III and PRES occurrence (P = 0.02). Additionally, acute graft versus host disease (aGVHD) occurred in 80% and 44.7% of patients in the PRES and non-PRES group, respectively (P = 0.001). The results of the Kaplan-Meier analysis revealed that the 5-year overall survival (OS) was 75% in the PRES group versus 92% in the non-PRES group was statistically significant between the two groups (P value < 0.0001). Our results indicated the diagnosis of PRES should be considered in β-MT pediatric patients.