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

Br J Haematol. 2021 Jun;193(6):1178-1184. doi: 10.1111/bjh.17398. Epub 2021 Mar 25.

Abstract

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-Münster 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.

Keywords: central nervous system; outcome; post-transplant lymphoproliferative disorder; transplant; treatment.

Publication types

  • Clinical Trial
  • Multicenter Study

MeSH terms

  • Adolescent
  • Adult
  • Allografts
  • Brain Neoplasms* / drug therapy
  • Brain Neoplasms* / etiology
  • Brain Neoplasms* / mortality
  • Child
  • Child, Preschool
  • Disease-Free Survival
  • Female
  • Hematopoietic Stem Cell Transplantation / adverse effects*
  • Humans
  • Infant
  • Injections, Spinal
  • Lymphoproliferative Disorders* / drug therapy
  • Lymphoproliferative Disorders* / etiology
  • Lymphoproliferative Disorders* / mortality
  • Male
  • Organ Transplantation / adverse effects*
  • Rituximab / administration & dosage*
  • Survival Rate

Substances

  • Rituximab