Primary post-transplant lymphoproliferative disorder of the central nervous system: characteristics, management and outcome in 25 paediatric patients

• Authors: TAJ M.M.; MAECKER-KOLHOFF B.; LING R.; BOMKEN S.; BURKHARDT B.; CHIANG A.K.S.; CSOKA M.; FUREDER A.; HAOUY S.; LAZIC J.; MIAKOVA N.; MINARD-COLIN V.; TURNER Suzanne Dawn; UYTTEBROECK A.; ATTARBASCHI A.
• Year of publication: 2021
• Type: Article in Periodical
• Magazine / Source: British journal of haematology
• MU Faculty or unit: Central European Institute of Technology
• Web: https://onlinelibrary.wiley.com/doi/10.1111/bjh.17398
• Doi: http://dx.doi.org/10.1111/bjh.17398
• Keywords: central nervous system; outcome; post- transplant lymphoproliferative disorder; transplant; treatment
• Description: Primary central nervous system (CNS) post-transplant lymphoproliferative disorder (PTLD) in childhood is rare. Twenty-five patients were retrieved from nine European Intergroup for Childhood Non-Hodgkin's Lymphoma and/or international Berlin-Frankfurt-Munster Study Group members. Types of allografts included kidney (n = 11), liver (n = 4), heart (n = 5), bowel (n = 1) and haematopoietic stem cells (n = 4). Eighteen were male, 16 >= 10 years old, 21 had monomorphic disease and 24 solid intracranial tumour masses. Four-year event-free and overall survival rates were 50% +/- 10% and 74% +/- 9% respectively. This report represents the largest paediatric series of CNS PTLD reported to date, showing favourable survival odds following systemic and intrathecal chemotherapy and rituximab administration.