Brain magnetic resonance angiography in splenectomized adults with β-thalassemia intermedia

Abstract

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.

Hypercoagulability in β-thalassemia: a status quo

“…In the same cohort of patients, it was noted that 27.6% had evidence of arterial stenosis on magnetic resonance angiography. However, there was no association between large-vessel stenosis on magnetic resonance angiography and silent strokes identified on MRI, leaving small arteriolar pathology as the most likely explanation for these silent lesions [49]. This is further confirmed through studies evaluating transcranial Doppler ultrasonography velocities in TI patients [50].…”

-Thalassemia Intermedia: A Clinical Perspective

“…Recent studies, to be further illustrated in the subsequent section, have also documented a high incidence of silent brain infarction, large cerebral vessel disease, and decreased neuronal function, primarily in the temporal and parietal lobes, among splenectomized patients with TI Taher et al 2010d;Musallam et al 2011aMusallam et al , 2012a. Large-vessel cerebrovascular disease significantly correlated with higher NTBI levels (Musallam et al 2011a), and decreased neuronal function was observed more frequently in patients with elevated LIC (.15 mg Fe/g dw) (Musallam et al 2012a).…”

Non-transfusion-dependent thalassemias

“…Recent studies have also documented a high prevalence of large cerebral vessel disease (magnetic resonance angiography) and decreased neuronal function (positron emission tomography-computed tomography) primarily in the temporal and parietal lobes in similar patient cohorts. [66][67][68] A significant association between the occurrence of these abnormalities and elevated iron overload indices was noted. 67,68 In the general population, and in patients with sickle cell disease, these silent cerebrovascular abnormalities are associated with subsequent risk of overt stroke and neurocognitive decline, further highlighting the importance of these findings.…”

“…[66][67][68] A significant association between the occurrence of these abnormalities and elevated iron overload indices was noted. 67,68 In the general population, and in patients with sickle cell disease, these silent cerebrovascular abnormalities are associated with subsequent risk of overt stroke and neurocognitive decline, further highlighting the importance of these findings. 66 Another vascular complication of NTDT (primarily β-thalassemia intermedia and hemoglobin E/β-thalassemia) that was found to occur at a relatively high frequency compared to patients with β-thalassemia major is pulmonary hypertension.…”