title: Cerebral vascular abnormalities in pediatric patients with sickle cell disease after hematopoietic cell transplant.
abstract: BACKGROUND
  Stroke is a common sequela of sickle cell disease (SCD). Patients with SCD who undergo hematopoietic stem cell transplantation (HSCT) with successful engraftment will not experience sickling. This ameliorates one aspect of stroke risk; however, the significance of preexisting cerebrovascular abnormalities remains unclear.
  
  
  METHODS
  We performed a literature search for neurological outcomes following HSCT for SCD. We searched for relevant neuroimaging and neurosurgical protocols. We identified 4 unique studies encompassing 196 patients. Of these, 81 had a history of a stroke, transient ischemic attack (TIA), cognitive dysfunction or cerebrovascular abnormalities identified by pretransplant neuroimaging, achieved stable engraftment, and had long-term follow-up.
  
  
  RESULTS
  Of the 81 patients, 1 had peritransplant (10 days prior transplant to 50 days posttransplant) TIA. One had posttransplant TIA within 36 to 72 months. None had strokes. Forty-five underwent cerebral imaging at nonuniform intervals. Among this group, 32 (71%) had stable cerebrovascular abnormalities on imaging, 6 (13%) had improvement, and 7 (16%) showed worsening.
  
  
  CONCLUSIONS
  Cerebrovascular abnormalities identified on neuroimaging may stabilize, improve, or worsen in patients after successful HSCT. Some patients may have neurological events such as TIA. Neurological outcomes in children with SCD post-HSCT have been inadequately studied.
authors: *Department of Pediatric Hematology and Oncology, Akron Children's Hospital, Akron, OH †Department of Pediatrics, Case Western Reserve University, and Rainbow Babies and Children's Hospital, Cleveland, OH.
authors: Prasad
authors: P
authors: Bodas
authors: Seth
authors: S
authors: Rotz
mesh_terms: D000755:Anemia, Sickle Cell / Q000150:complications*; D002561:Cerebrovascular Disorders / Q000175:diagnosis / Q000209:etiology*; D003072:Cognition Disorders / Q000175:diagnosis / Q000209:etiology*; D015331:Cohort Studies; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects*; D006801:Humans; D007091:Image Processing, Computer-Assisted; D011379:Prognosis; D012196:Review Literature as Topic