Hellstern P, Mannhalter C, Köhler M, Kiehl R, von Blohn G, Wenzel E, Deutsch E
Haemostasis. 1985;15(3):215-9. doi: 10.1159/000215147.
A 12-year-old girl with lifelong hemorrhagic episodes was found to have both a dys-form of homozygous factor XI deficiency and heterozygous factor XII deficiency. The heredity of the coagulation defects was confirmed by family studies. Severe bleeding after dental surgery occurred in spite of replacement therapy and local measures including fibrin glue. Our findings suggest that the risk of bleeding in patients with homozygous factor XI deficiency must not be underestimated and that the most effective measure is the transfusion of sufficient amounts of fresh frozen plasma until at least the 5th postoperative day.
一名有终生出血发作史的12岁女孩被发现同时存在纯合子因子XI缺乏的异常形式和杂合子因子XII缺乏。通过家族研究证实了凝血缺陷的遗传性。尽管进行了替代治疗以及包括纤维蛋白胶在内的局部措施，但牙科手术后仍发生了严重出血。我们的研究结果表明，纯合子因子XI缺乏患者的出血风险绝不能被低估，最有效的措施是输注足够量的新鲜冰冻血浆，至少持续到术后第5天。
