Cerebral infarction in β-thalassemia intermedia: breaking the silence

Despite remarkable advances in understanding cerebrovascular disease attributed to sickle cell anemia, data from other hemoglobinopathies have only recently started to emerge. Several brain magnetic resonance imaging studies confirm a high prevalence of silent ischemic lesions in patients with β-thalassemia intermedia, especially in splenectomized adults who are transfusion-independent and those with elevated platelet counts. Large-vessel disease is also common in this patient population but without apparent association with silent white matter infarcts, leaving smaller arteriolar involvement as a potential explanation. The hypothesized pathophysiology is multifactorial with hypercoagulability and toxicity from free iron species playing major roles. The long-term sequelae of such covert findings is unknown, although experience from patients with sickle cell anemia confirms their association with subsequent overt stroke and neurocognitive deficits. The roles of transfusion and antiplatelet therapy to prevent the occurrence and progression of silent ischemic lesions in patients with β-thalassemia intermedia should be the focus of future trials.