Beta (β)‐thalassemia and sickle cell disease (SCD) are characterized by a hypercoagulable state, which can significantly influence organ complication and disease severity. While red blood cells (RBCs) and erythroblasts continue to play a central role in the pathogenesis of thrombosis in β‐thalassemia and SCD, additional factors such as free heme, inflammatory vasculopathy, splenectomy, among other factors further contribute to the complexity of thrombotic risk. Thus, understanding the role of the numerous factors driving this hypercoagulable state will enable healthcare practitioners to enhance preventive and treatment strategies and develop novel therapies for the future. We herein describe the pathogenesis of thrombosis in patients with β‐thalassemia and SCD. We also identify common mechanisms underlying the procoagulant profile of hemoglobinopathies translating into thrombotic events. Finally, we review the currently available prevention and clinical management of thrombosis in these patient populations.

中文翻译：

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血红蛋白病中的高凝状态：解码血栓形成威胁


β （β） - 地中海贫血和镰状细胞病 （SCD） 的特征是高凝状态，这会显着影响器官并发症和疾病严重程度。虽然红细胞 （RBC） 和成红细胞继续在β地中海贫血和 SCD 血栓形成的发病机制中发挥核心作用，但游离血红素、炎性血管病变、脾切除术等其他因素进一步导致血栓形成风险的复杂性。因此，了解导致这种高凝状态的众多因素的作用将使医疗保健从业者能够加强预防和治疗策略，并为未来开发新的疗法。我们在此描述了 β 地中海贫血和 SCD 患者血栓形成的发病机制。我们还确定了血红蛋白病促凝特征转化为血栓形成事件的常见机制。最后，我们回顾了目前可用的这些患者群体血栓形成的预防和临床管理。