Abstract
Patients with non-transfusion-dependent thalassemia (NTDT) are at risk of developing brain ischemia. Transcranial Doppler (TCD) has been established as a useful screening tool of cerebrovascular disease in patients with sickle cell disease. Proteins neuron specific enolase (NSE) and S100B are biomarkers that reflect CNS injury. The purpose of this study is to evaluate cerebral vessel vasculopathy and brain damage in NTDT patients using non-invasive methods as TCD and measurement serum levels of NSE and S100B. We included in our study 30 patients with NTDT, aged between 8 and 62 years old (mean: 29.4, median: 32) who presented in our Unit for regular follow-up. We performed in all patients a non-imaging TCD examination and have measured serum S100, NSE and lactate dehydrogenase (LDH) levels. We investigated the possible correlation between TCD results and S100B, NSE and LDH levels as well as between NSE-LDH and S100B-LDH levels by regression analysis. We found a statistically significant relationship for both NSE, S100B with LDH. We also found a statistically significant relationship for S100B and time-averaged mean velocity (TAMV)/peak velocity of left middle cerebral artery (MCA), NSE and pulsatility index (PI)/resistive index (RI) of the left posterior cerebral artery (PCA). TCD results correlated with biomarkers for brain ischemia. This finding enhances the role of TCD as a screening tool for brain ischemia in patients with NTDT.
Highlights
The term non-transfusion-dependent thalassemia (NTDT) refers to patients with thalassemia but without need of regular transfusions even though they may require occasional or even frequent transfusions in certain clinical conditions and for defined periods of time
Patients with NTDT have 4.38 times a greater risk of developing a thromboembolic event compared to β-thalassemia major patients as reported in a recent study on 8860 patients with β-thalassemia major and intermedia [3]
The purpose of this study is to evaluate cerebral vessel vasculopathy and brain damage in patients with NTDT using non-invasive methods as Transcranial Doppler (TCD) and measurement of neuron specific enolase (NSE), S100 calcium-binding protein B (S100B) and lactate dehydrogenase (LDH) in blood serum
Summary
The term non-transfusion-dependent thalassemia (NTDT) refers to patients with thalassemia but without need of regular transfusions even though they may require occasional or even frequent transfusions in certain clinical conditions and for defined periods of time. It includes: β-thalassemia intermedia, α-thalassemia intermedia and hemoglobin E/β-thalassemia [1]. Recent studies show that patients with NTDT are prone to suffer thromboembolic events. Ischemic strokes consist a major complication in patients with NTDT and can be divided in two categories: overt strokes and silent infarcts. In contrast to an overt stroke a silent infarct is not associated to a neurologic abnormality and cannot be detected on clinical examination [4]
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