Department of Internal Medicine, Hematology-Oncology Division, American University of Beirut Medical Centre, Beirut, Lebanon.
Mediterr J Hematol Infect Dis. 2011;3(1):e2011025. doi: 10.4084/MJHID.2011.025. Epub 2011 May 25.
Although the life expectancy of thalassemia patients has markedly improved over the last few decades, patients still suffer from many complications of this congenital disease. The presence of a high incidence of thromboembolic events, mainly in thalassemia intermedia, has led to the identification of a hypercoagulable state in these patients. In this review, the molecular and cellular mechanisms leading to hypercoagulability in thalassemia are highlighted, with a special focus on thalassemia intermedia being the group with the highest incidence of thrombotic events as compared to other types of thalassemia. Clinical experience and available clues on optimal management are also discussed.
尽管过去几十年间地中海贫血患者的预期寿命显著提高，但他们仍饱受这种先天性疾病的多种并发症困扰。极高的血栓栓塞事件发生率（主要发生于中间型地中海贫血）使得人们发现这些患者处于高凝状态。在这篇综述中，我们重点阐述了导致地中海贫血高凝的分子和细胞机制，并特别关注中间型地中海贫血，因为与其他类型的地中海贫血相比，该类型的血栓栓塞事件发生率最高。我们还讨论了临床经验和关于最佳治疗方法的现有线索。
