IRCCS Ca' Granda Foundation Maggiore Policlinico Hospital, University of Milan, Via Francesco Sforza 35, Milan, Italy.
Blood Rev. 2012 Apr;26 Suppl 1:S20-3. doi: 10.1016/S0268-960X(12)70007-3.
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.
β-地中海贫血的特征是呈高凝状态和血栓形成风险增加，这可能导致严重的发病率和死亡率。导致高凝状态的分子和细胞机制多种多样，包括慢性血小板激活、红细胞膜改变、血管内皮细胞上黏附分子的异常表达以及止血功能失调。定期输血可降低血栓形成的风险，而脾切除术则显著增加风险。非输血依赖型地中海贫血的脾切除成人也存在发生缺血性脑损伤的高风险。应考虑降低血栓形成风险的策略，包括输血治疗以提高血红蛋白水平和避免或延迟脾切除术。
