Peter M K, Meili E O, von Felten A
Gerinnungslabor, Departement für Innere Medizin, Universitätsspital Zürich.
Schweiz Med Wochenschr. 1996 Jun 8;126(23):999-1005.
It is well known that the extent of bleeding in patients with a deficiency of factor XI does not parallel the residual factor activity. Therefore, additional hemostatic aspects must be taken into consideration. 27 patients of 18 different families--6 with severe (FXI < 0.01 to 0.09 U/ml) and 21 with moderate (FXI 0.20 to 0.252 U/ml) factor XI deficiency--were reinvestigated regarding hemostasis including bleeding time and platelet function. 16 had an enhanced bleeding tendency, while the others--including 3 with severe FXI deficiency of < 0.01 U/ml--never suffered from bleeding complications despite delicate surgery in some. All 16 of the symptomatic patients had, besides the reduction of factor XI activity to various extents, an additional hemostatic defect: 3 had a moderate alpha/delta storage pool detect of the platelets, 11 a platelet anomaly mainly characterized by reduced ADP aggregation and 2 an isolated prolonged bleeding time of unknown cause. Synthesis of platelet thromboxane was unimpaired in all patients as tested by formation of malondialdehyde after stimulation with N-ethyl maleimide. Hence, in patients with a known factor XI deficiency diagnostic and prophylactic measures before surgery should concentrate on such additional findings. Preoperative administration of desmopressin, and not replacement of factor XI, will be the treatment of choice in these cases.
众所周知，缺乏因子XI的患者出血程度与残余因子活性并不平行。因此，必须考虑其他止血方面。对来自18个不同家庭的27例患者进行了重新研究，其中6例为严重因子XI缺乏（FXI<0.01至0.09 U/ml），21例为中度因子XI缺乏（FXI 0.20至0.252 U/ml），研究内容包括止血情况，如出血时间和血小板功能。16例患者有出血倾向增加，而其他患者——包括3例FXI严重缺乏（<0.01 U/ml）的患者——尽管有些人接受了精细手术，但从未出现出血并发症。所有16例有症状的患者，除了因子XI活性不同程度降低外，还有额外的止血缺陷：3例有中度血小板α/δ储存池缺陷，11例有血小板异常，主要表现为ADP聚集减少，2例有原因不明的单纯性出血时间延长。经N-乙基马来酰亚胺刺激后通过丙二醛形成测试，所有患者的血小板血栓素合成均未受损。因此，对于已知因子XI缺乏的患者，手术前的诊断和预防措施应集中在这些额外的发现上。在这些情况下，术前给予去氨加压素而非补充因子XI将是首选治疗方法。
