title: Brain magnetic resonance angiography in splenectomized adults with β-thalassemia intermedia.
abstract: BACKGROUND
  Hypercoagulability and venous thromboembolism are common in patients with β-thalassemia intermedia (TI), especially in the splenectomized adult. Although arterial involvement is not commonly reported, we have recently observed a high prevalence (60%) of silent brain infarction on brain MRI in 30 splenectomized adults with TI. The pathophysiology of these white matter lesions remains unknown.
  
  
  METHODS
  In this prospective work, we evaluated magnetic resonance angiography (MRA) scans of the same cohort of 30 patients. Data collected were the presence or absence of vascular lesions, their locations, and severity. Correlations between MRA abnormality and patients/disease characteristics were evaluated. Comparisons between MRA and previous MRI findings were made.
  
  
  RESULTS
  Of 29 evaluable patients, 8 (27.6%) had evidence of arterial stenosis on MRA. The majority of lesions had mild narrowing and mostly involved the internal carotid artery. Five patients (17.2%) had evidence of aneurysms. Low total hemoglobin and high non-transferrin-bound iron levels independently characterized patients with evidence of stenosis on MRA. Among the 18 patients with silent brain infarction on MRI, three had evidence of stenosis on MRA with only one patient having lesions that could explain the silent infarcts.
  
  
  CONCLUSIONS
  Cerebral vasculopathy is common in splenectomized adults with TI. However, large-vessel disease does not explain the occurrence of silent brain infarction. The combined use of MRA and MRI better identifies splenectomized TI adults with neuroimaging abnormalities.
authors.affiliation: Division of Hematology & Oncology, Department of Internal Medicine, American University of Beirut Medical Center.
authors.forename: Khaled M
authors.initials: KM
authors.lastname: Musallam
authors.forename: Ahmad
authors.initials: A
authors.lastname: Beydoun
authors.forename: Roula
authors.initials: R
authors.lastname: Hourani
authors.forename: Wassim
authors.initials: W
authors.lastname: Nasreddine
authors.forename: Roy
authors.initials: R
authors.lastname: Raad
authors.forename: Suzanne
authors.initials: S
authors.lastname: Koussa
authors.forename: Ali T
authors.initials: AT
authors.lastname: Taher
mesh_terms: D000328:Adult; D001921:Brain / Q000000981:diagnostic imaging*; D006801:Humans; D018810:Magnetic Resonance Angiography / Q000379:methods*; D008958:Models, Molecular; D011446:Prospective Studies; D011859:Radiography; D013156:Splenectomy; D014463:Ultrasonography; D017086:beta-Thalassemia / Q000000981:diagnostic imaging*