Asymptomatic brain magnetic resonance imaging abnormalities in splenectomized adults with thalassemia intermedia

Abstract

Background: A high incidence of thrombotic events in thalassemia intermedia (TI) patients led to the identification of a hypercoagulable state. Brain involvement has not been widely studied in TI, although limited reports confirm a low incidence of overt stroke and high incidence of silent brain infarcts. Patients/methods: This was a prospective study conducted on 30 adult, splenectomized TI patients. Patients were screened for absence of neurological signs or symptoms, and stroke-related risk factors. Patient charts were reviewed for demographics, duration since splenectomy, and any history of transfusion therapy. Blood samples were obtained for complete blood counts and serum ferritin. Direct determination of liver iron concentration (LIC) was performed by R2 magnetic resonance imaging (MRI). Brain MRI was performed on all patients, looking for ischemic lesions and/or atrophy. Results: The mean age of patients was 32.1 ± 11 years (range, 18-54 years), with a male to female ratio of 13:17. Eighteen patients (60%) had evidence of one or more white matter lesions (WMLs) on brain MRI, all involving the subcortical white matter. Fourteen patients had evidence of multiple WMLs, with a mean of 5 ± 10 lesions (range, 2 to > 40 lesions). The vast majority of patients (94%) had small (< 0.5 cm) to medium (0.5-1.5 cm) WMLs, with only one patient showing evidence of a large (> 1.5 cm) WML. Eleven patients (37%) had mild cerebral atrophy. On multivariate analysis only age and transfusion history were independently and significantly associated with the occurrence of zero, single or multiple WMLs. Conclusion: WMLs and brain atrophy are a common finding in adult, splenectomized, TI patients. Increasing age and transfusion naivety are associated with a higher incidence and multiplicity of lesions.

Hypercoagulability in β-thalassemia: a status quo

“…Most patients had evidence of multiple lesions, mainly in the frontal subcortical white matter, followed by the parietal and occipital subcortical white matter [48]. In these cases, increasing age and transfusion naivety were both independently associated with a higher occurrence and multiplicity of lesions [48]. In the same cohort of patients, it was noted that 27.6% had evidence of arterial stenosis on magnetic resonance angiography.…”

“…In 18 patients (60%), one or more ischemic lesions were detected in the subcortical white matter on brain MRI. Most patients had evidence of multiple lesions, mainly in the frontal subcortical white matter, followed by the parietal and occipital subcortical white matter [48]. In these cases, increasing age and transfusion naivety were both independently associated with a higher occurrence and multiplicity of lesions [48].…”

Non-transfusion-dependent thalassemias

“…24,83,84 Observational studies continue to confirm that NTDT patients who receive transfusions experience fewer leg ulcers, thrombotic events, pulmonary hypertension, and silent brain infarcts compared with transfusion-naïve patients. 30,64,80,85,86 Successful management of the hematopoietic compensatory extramedullary pseudotumors has also been reported using transfusion therapy with and without radiation or surgery, especially in the most debilitating cases with paraspinal involvement. 31 It is absolutely essential to assess the patient carefully over the first few months after the diagnosis is established and not to embark on any treatment modality, especially transfusion therapy, too hastily.…”

Associated risk factors for silent cerebral infarcts in sickle cell anemia: low baseline hemoglobin, sex, and relative high systolic blood pressure

“…21,22 Although the etiology of SCI is unknown, the consistent finding that anemia is a risk factor strongly implicates cerebral hemodynamic insufficiency as a central component. All patients with chronic anemia have a compensatory mechanism in the brain (auto-regulation) that occurs with vasodilation of the cerebral vasculature and manifests initially as increased cerebral blood flow.…”