β-地中海贫血患者血栓栓塞事件和神经系统损害的研究进展

Negin Kheiri, Morteza Zangeneh Soroush, Marzieh Aboutorabi
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引用次数: 0

摘要

Β-thalassemia是一种严重的遗传性疾病,它会减少血红蛋白的合成。地中海贫血综合征(包括β-地中海贫血重度和中度)的主要并发症之一是血栓栓塞事件。此外,血栓栓塞事件在非输血依赖型地中海贫血中比输血依赖型β-地中海贫血中更常见。包括红细胞和血小板异常、抗凝血酶III、蛋白C和蛋白S以及脾切除术在内的高凝状态的组合与血栓形成事件有关,但这些患者可以通过输血、羟脲、抗凝剂和阿司匹林来预防和治疗血栓形成事件。此外,最近的研究表明β-地中海贫血患者的脑部病变也参与其中。β-地中海贫血中脑血管事件受累率为29-83%,而健康人无症状脑病变发生率为0-11%。此外,慢性缺氧、铁超载、骨髓扩张和去铁胺神经毒性等神经系统并发症已被归因于各种因素。本文综述了β-地中海贫血患者的血栓栓塞事件和神经病变及其可能的治疗方法。
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A review on thromboembolic events and neurological lesions in patients with β-thalassemia
Β-thalassemia is the severe form of genetic illnesses which decreases the hemoglobin synthesis. One of the major complications in thalassemic syndromes, including β-thalassemia major and intermedia is thromboembolic events. In addition, thromboembolic events are more common in non-transfusion-dependent thalassemia than those in well-transfusion-dependent β-thalassemia. A combination of hypercoagulable states including, abnormalities in red blood cells, and platelet, antithrombin III, protein C, and protein S, and splenectomy are involved in thrombotic events, but thromboembolic events can be prevented and treated in these patients via blood transfusion, hydroxyurea, anticoagulants, and aspirin. Moreover, recent studies have demonstrated the involvement of the brain lesion in β-thalassemia patients. The involvement of vascular events of brain in patients with β-thalassemia intermedia is 29-83%, but the rate of asymptomatic brain lesions in the healthy people is 0-11%. In addition, neurological complications which have been attributed to various factors are chronic hypoxia, iron overload, bone marrow expansion, and desferrioxamine neurotoxicity. This review evaluated thromboembolic events and neurological lesions in patients with β-thalassemia and its probable curative therapy.
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来源期刊
CiteScore
0.80
自引率
33.30%
发文量
33
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