: Posterior reversible encephalopathy syndrome (PRES) is a rare but potentially life-threatening neurological complication following paediatric allogeneic haematopoietic stem cell transplantation (HSCT). Its heterogeneous presentation and the absence of standardized guidance contribute to variability in diagnosis and management. The aim was to develop multidisciplinary, consensus-based recommendations for the diagnosis, management and follow-up of PRES in paediatric allogeneic HSCT recipients. A modified Delphi process was conducted between February and September 2025. An international panel comprising transplant physicians, neurologists, neuro-oncologists and neuroradiologists participated in four iterative rounds. A systematic literature review informed statement development across five domains: pathogenesis, clinical features, diagnostics, management and follow-up. Predefined agreement thresholds determined consensus. Thirty-eight statements achieved consensus in the first round, indicating strong agreement. Key recommendations addressed risk factors, early clinical recognition, appropriate neuroimaging and management of contributory factors including hypertension and immunosuppressive therapy. Emphasis was placed on multidisciplinary care, prompt diagnosis and tailored management. Guidance on neurological monitoring and long-term follow-up was also established. These consensus recommendations provide a standardized framework for the management of PRES in paediatric allogeneic HSCT recipients. Their implementation may improve early recognition, optimize treatment and enhance outcomes, while highlighting priorities for future research and prospective guideline development.
Leardini, D., Bergonzini, L., Muratore, E., Pace, V., Cacace, F., Barrui, V., et al. (2026). Consensus recommendations on diagnosis and management of posterior reversible encephalopathy syndrome in paediatric haematopoietic stem cell transplantation. BRITISH JOURNAL OF HAEMATOLOGY, 00, 1-12 [10.1111/bjh.70873].
Consensus recommendations on diagnosis and management of posterior reversible encephalopathy syndrome in paediatric haematopoietic stem cell transplantation
Leardini, Davide;Bergonzini, Luca;Muratore, Edoardo;Pace, Valentina;Fetta, Anna;Toni, Francesco;Masetti, Riccardo;Cordelli, Duccio Maria
2026
Abstract
: Posterior reversible encephalopathy syndrome (PRES) is a rare but potentially life-threatening neurological complication following paediatric allogeneic haematopoietic stem cell transplantation (HSCT). Its heterogeneous presentation and the absence of standardized guidance contribute to variability in diagnosis and management. The aim was to develop multidisciplinary, consensus-based recommendations for the diagnosis, management and follow-up of PRES in paediatric allogeneic HSCT recipients. A modified Delphi process was conducted between February and September 2025. An international panel comprising transplant physicians, neurologists, neuro-oncologists and neuroradiologists participated in four iterative rounds. A systematic literature review informed statement development across five domains: pathogenesis, clinical features, diagnostics, management and follow-up. Predefined agreement thresholds determined consensus. Thirty-eight statements achieved consensus in the first round, indicating strong agreement. Key recommendations addressed risk factors, early clinical recognition, appropriate neuroimaging and management of contributory factors including hypertension and immunosuppressive therapy. Emphasis was placed on multidisciplinary care, prompt diagnosis and tailored management. Guidance on neurological monitoring and long-term follow-up was also established. These consensus recommendations provide a standardized framework for the management of PRES in paediatric allogeneic HSCT recipients. Their implementation may improve early recognition, optimize treatment and enhance outcomes, while highlighting priorities for future research and prospective guideline development.| File | Dimensione | Formato | |
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