Beta (β)-thalassemia is characterized by a hypercoagulable state and an increased risk of thrombosis, which can result in significant morbidity and mortality. The molecular and cellular mechanisms contributing to hypercoagulability are diverse and include chronic platelet activation, alteration of red blood cell membranes, abnormal expression of adhesion molecules on vascular endothelial cells, and dysregulation of hemostasis. Regular transfusions decrease the risk of thrombosis, whereas splenectomy significantly increases the risk. Splenectomized adults with non-transfusion-dependent thalassemia are also at high risk for ischemic brain damage. Strategies to lower the risk of thrombosis should be considered, including transfusion therapy to raise hemoglobin levels and avoidance or delay of splenectomy.

中文翻译：

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非输血依赖型地中海贫血的高凝性。

β（β）地中海贫血的特征在于高凝状态和血栓形成的风险增加，这可能导致明显的发病率和死亡率。促成高凝的分子和细胞机制是多种多样的，包括慢性血小板活化，红细胞膜改变，血管内皮细胞上粘附分子的异常表达以及止血失调。定期输血降低了血栓形成的风险，而脾切除术则大大增加了风险。非输血依赖型地中海贫血的脾切除成人也有缺血性脑损伤的高风险。应考虑降低血栓形成风险的策略，包括提高血红蛋白水平的输血治疗以及避免或延迟脾切除术。