Abstract Number: PB0087
Meeting: ISTH 2020 Congress
Background: Despite advances in understanding silent cerebral ischemia (SCI) in sickle cell anemia, data from thalassemia has only recently started to emerge. Cerebral venous and arterial thromboembolism have been reported both in non-transfusion and transfusion-dependent thalassemia (NTDT and TDT).
Aims: We highlight advances in understanding the prevalence of SCI in thalassemia, discuss the potential underlying risk factors, and determine required efforts to achieve optimal prevention and treatment.
Methods: Several studies have been conducted on 135 neurologically intact thalassemia patients (49 NTDT and 86 TDT) from 2008 to 2018. Diffusion-weighted imaging brain MRI (1.5 tesla) was performed.
Results: The microthrombosis in the brain in asymptomatic patients with NTDT and TDT has been shown changes in the white matter suggestive of ischemia and small infarctions (figure1).
Overall, white matter lesions (WMLs) was found in 30 of 135 TDT and NTDT patients (22.2%) including 11 of 49 NTDT (22.4%) and 19 of 86 TDT (22%). Splenectomy, age >30 years old, high serum ferritin levels, platelet counts > 500000/mm3 and hemoglobin level < 9 g/dl are risk factors for SCI.
In another study, brain MRI in 35 NTDT and TDT patients was evaluated over 3 years. The frequency of WMLs was increased from 10 patients (28.6%) in the first MRI to 13 patients (37.1%) in the second MRI after 3 years.
Conclusions: The long-term sequelae of such covert findings are unknown, although experience from patients with sickle cell anemia confirms their association with subsequent overt stroke and neurocognitive deficits. The high-risk patients with thrombocytosis, splenectomy, severe iron overload, and older age should be under close follow-up and evaluated regularly as well as brain MRI. Aspirin, keep the Hb levels ≥9 g/dL, regular iron chelation regimen and try to preserve the spleen are the management strategies to prevent new or progressive silent brain lesions.
To cite this abstract in AMA style:Karimi M, Haghpanah S. Silent Brain Ischemia in Thalassemia Patients: Breaking the Silence [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/silent-brain-ischemia-in-thalassemia-patients-breaking-the-silence/. Accessed January 28, 2022.
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