Calcineurin inhibitor-free strategies for prophylaxis and treatment of GVHD in children with posterior reversible encephalopathy syndrome after stem cell transplantation

Vered Shkalim-Zemer, Osnat Konen, Yoel Levinsky, Orli Michaeli, Anat Yahel, Aviva Krauss, Isaac Yaniv, Jerry Stein*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

Abstract

Background: Posterior reversible encephalopathy syndrome (PRES) is a distinct clinico-radiologic entity that can occur following allogeneic hematopoietic stem cell transplantation, often in the context of treatment with calcineurin inhibitors (CNIs). Procedure: We describe the results of CNI-free management of 14 children with PRES and review the clinical and radiologic manifestations of their presentation. Results: Discontinuation of CNIs usually resulted in remission of PRES, but patients with established graft versus host disease (GVHD) at the time when treatment was changed often experienced progressive GVHD despite administration of immune suppressive and modulating treatments. All but three patients experienced full neurologic recovery. Nine children died as a result of either GVHD, disease relapse, or severe infection. Conclusions: Discontinuation of CNIs results in neurologic improvement in most cases, but superior alternative immune modulatory treatment is needed to prevent progression of established GVHD.

Original languageEnglish
Article numbere26531
JournalPediatric Blood and Cancer
Volume64
Issue number11
DOIs
StatePublished - Nov 2017

Keywords

  • GVHD
  • PRES
  • calcineurin inhibitor-free

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