Sherer Y, Bar-Zohar D, Levy Y, Shoenfeld Y
Department of Medicine B, Chaim Sheba Medical Center, Tel-Hashomer, Israel.
Ann Med Interne (Paris). 1998 Dec;149(8):492-4.
Factor XI deficiency is an hereditary coagulopathy that is usually associated with milder tendency to bleeding with comparison to hemophilia A. While the failure of stable fibrin clot formation may lead to bleeding, it is speculated that the same process may provide a protection against thrombosis of injured arteries due to atherosclerotic plaque rupture. Whereas 2 studies indicate that hemophiliacs have decreased mortality rate from cardiovascular diseases, there is no similar data regarding factor XI deficiency patients. In here we report about 3 patients with severe factor XI deficiency who have a long-standing history of thromboembolic phenomena: 2 patients with myocardial infarctions, and one patient with transient ischemic attacks. We discuss the possible role of factor XI in thrombosis, and whether its deficiency may protect patients from thromboembolic phenomena.
因子XI缺乏症是一种遗传性凝血病，与A型血友病相比，通常伴有较轻的出血倾向。虽然稳定纤维蛋白凝块形成失败可能导致出血，但据推测，同一过程可能对动脉粥样硬化斑块破裂所致损伤动脉的血栓形成起到保护作用。有两项研究表明血友病患者心血管疾病死亡率降低，但关于因子XI缺乏症患者尚无类似数据。在此，我们报告3例严重因子XI缺乏症患者，他们有长期的血栓栓塞现象病史：2例心肌梗死患者和1例短暂性脑缺血发作患者。我们讨论了因子XI在血栓形成中的可能作用，以及其缺乏是否能保护患者免受血栓栓塞现象的影响。
