Consensus recommendations on diagnosis and management of posterior reversible encephalopathy syndrome in paediatric haematopoietic stem cell transplantation

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.

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Publication Details

Journal
British Journal of Haematology
Published
2026-11-01
DOI
https://doi.org/10.1111/bjh.70873
Primary Topic
Neurological Complications and Syndromes
Type
article
Field-Weighted Citation Impact
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article

Consensus recommendations on diagnosis and management of posterior reversible encephalopathy syndrome in paediatric haematopoietic stem cell transplantation

Luca De Palma, Angela Mastronuzzi, Francesco Paolo Paolo Tambaro, Luca Bergonzini et al.
British Journal of Haematology
Neurological Complications and Syndromes
article

Consensus recommendations on diagnosis and management of posterior reversible encephalopathy syndrome in paediatric haematopoietic stem cell transplantation

Luca De Palma, Angela Mastronuzzi, Francesco Paolo Paolo Tambaro, Luca Bergonzini, Davide Leardini, Domenico Tortora, Simone Cesaro, Fabiana Cacace, Giorgio Costagliola, Duccio Maria Cordelli, Riccardo Masetti, Marco Zecca, Maura Faraci, Edoardo Muratore, Alessandro Ascani Orsini, Thomas Foiadelli, Gaia Alessandra Kullmann, Dario Pruna, Vanessa Barrui, Valentina Pace, Giovanna Lucchini, Stefano Sartori, Francesco Toni, Anna Fetta, Maria M Mancardi, Stefania Giovanna Colafati
article en

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.

British Journal of Haematology
Università Cattolica del Sacro Cuore (IT), University of Padua (IT), University of Cagliari (IT), Istituto Giannina Gaslini (IT), Azienda Ospedaliera Universitaria Integrata Verona (IT), Azienda Ospedaliera San Gerardo (IT), Istituto delle Scienze Neurologiche di Bologna (IT), Bambino Gesù Children's Hospital (IT), University Children's Hospital Zurich (CH), Azienda Ospedale - Università Padova (IT), Policlinico San Matteo Fondazione (IT), Istituti di Ricovero e Cura a Carattere Scientifico (IT), Azienda Ospedaliera Universitaria Pisana (IT), Azienda Ospedaliera G. Brotzu (IT), University of Bologna (IT)
Openalex Percentile: Top 12%
Neurological Complications and Syndromes
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