Haemophilia Unit, Haematology Department, Hospital Universitario La Paz, Paseo de la Castellana 261, Madrid, Spain.
Clin Appl Thromb Hemost. 2010 Apr;16(2):209-13. doi: 10.1177/1076029608327864. Epub 2008 Dec 1.
Factor XI deficiency is a rare disease found predominantly in Ashkenazi Jews. There is a poor correlation between factor XI level and bleeding in patients with factor XI deficiency. Individuals with severe factor XI deficiency are usually at risk of excessive bleeding after surgery and injury, particularly when trauma involves tissues rich in fibrinolytic activity. Women with partial or severe deficiency are at risk of excessive uterine bleeding during labor. The unpredictable nature of factor XI deficiency complicates management during pregnancy and delivery. This review gives an overview of the management of pregnant women with factor XI deficiency.
Factor XI 缺乏症是一种主要见于阿什肯纳兹犹太人的罕见疾病。Factor XI 缺乏症患者的 Factor XI 水平与出血之间相关性较差。严重缺乏 Factor XI 的个体在手术后和受伤后通常有过度出血的风险，特别是当创伤涉及富含纤维蛋白溶解活性的组织时。部分或严重缺乏的女性在分娩时存在子宫过度出血的风险。Factor XI 缺乏症的不可预测性质使妊娠和分娩期间的管理复杂化。本文综述了 Factor XI 缺乏症孕妇的管理。
