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 language | English (US) |
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Article number | e27912 |
Journal | Pediatric Blood and Cancer |
Volume | 66 |
Issue number | 10 |
DOIs | |
State | Published - 2019 |
Externally published | Yes |
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