Heim M U, Lutze G, Aumann V, Schumacher J, Freigang B
Institut für Transfusionsmedizin und Immunhämatologie mit Blutbank, Germany.
Klin Padiatr. 2002 May-Jun;214(3):128-31. doi: 10.1055/s-2002-30148.
The rare factor XI deficiency is associated with different profuse bleeding without correlation to the severity of reduction of factor XI. Accordingly, traumata or surgical procedures may cause unexpected excessive bleeding in asymptomatic patients. After surgery of a nine-year-old girl with factor XI deficiency (8 per cent) profuse bleeding occurred which could only be stopped after infusion of desmopressin. After administration the factor XI activity was increased to 31 per cent, the factor VIII even to 290 per cent over the normal range. We suppose that the favorable clinical effectiveness is not only related to the increasing factor XI activity but also to the elevation of the factor VIII/von-Willebrand-complex.
It is recommended to give desmopressin as firstline therapy of bleeding by factor XI deficiency since the only effective alternative such as substitution of factor XI by transfusion of fresh frozen plasma is associated with the risk of transmission of virus infections.
罕见的因子XI缺乏症与不同程度的大量出血相关，且与因子XI降低的严重程度无关。因此，创伤或外科手术可能在无症状患者中导致意外的过度出血。一名9岁因子XI缺乏症（8%）女童手术后发生大量出血，仅在输注去氨加压素后出血才停止。给药后，因子XI活性增加至31%，因子VIII甚至超过正常范围增至290%。我们推测良好的临床疗效不仅与因子XI活性增加有关，还与因子VIII/血管性血友病因子复合物升高有关。
建议将去氨加压素作为因子XI缺乏症出血的一线治疗药物，因为唯一有效的替代方法，如输注新鲜冷冻血浆替代因子XI，存在病毒感染传播的风险。
