Successful treatment with eculizumab for posterior reversible encephalopathy syndrome due to underlying transplant-associated thrombotic microangiopathy in patients transplanted for sickle cell disease

Nabanita Bhunia, Rolla Abu-Arja, Rajinder P.S. Bajwa, Jeffery J. Auletta, Hemalatha G. Rangarajan

Research output: Contribution to journalArticlepeer-review

4 Scopus citations

Abstract

Preexisting endothelial dysfunction and vascular injury sustained during allogeneic hematopoietic cell transplantation (HCT) increases risk for endothelial injury-related complications such as posterior reversible encephalopathy syndrome (PRES) and transplant-associated thrombotic microangiopathy (TA-TMA) in patients with sickle cell disease (SCD). We report two patients with SCD who developed PRES following allogeneic HCT. In both patients, PRES-related symptoms resolved only after a diagnosis of TA-TMA was established and eculizumab therapy was initiated. Renal manifestations at diagnosis included non-nephrotic range proteinuria and hypertension. This report highlights the importance of screening PRES-affected SCD HCT recipients for TA-TMA as usual treatment strategies may be inadequate.

Original languageEnglish (US)
Article numbere27912
JournalPediatric Blood and Cancer
Volume66
Issue number10
DOIs
StatePublished - 2019
Externally publishedYes

Keywords

  • eculizumab
  • hematopoietic cell transplant
  • posterior reversible encephalopathy syndrome
  • sickle cell disease
  • transplant-associated thrombotic microangiopathy

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

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