凝血因子Ⅺ缺乏症患者围手术期替代治疗

The alternative therapy of coagulation factor Ⅺ deficient patients during perioperative period

目的认识并探讨凝血因子Ⅺ缺乏症合并外科疾病,特别是合并巨大肿瘤的围手术期替代治疗。方法通过文献回顾学习、病例总结的方法,对2名凝血因子Ⅺ缺乏症患者,监控手术前、后APTT、Ⅺ浓度和TEG,输注新鲜冰冻血浆和辅以其他治疗。结果输注新鲜冰冻血浆后,凝血因子Ⅺ合并巨大肿瘤患者APTT由103.6 s缩短到46.5—57.1 s,凝血因子Ⅺ合并阑尾炎患者APTT由87.7 s缩短到42.8—46.6 s。合并肿瘤患者APTT控制在46.5 s后行手术治疗,术中未大量出血,术后APTT控制在46.5—57.1 s无明显渗血、渗液。合并阑尾炎患者APTT控制在46.6 s后行手术治疗,术中出血很少,术后APTT控制在42.8—46.6 s,无渗血、渗液。结论凝血因子Ⅺ缺乏合并外科疾病者围手术期新鲜冰冻血浆输注,可明显改善患者的凝血功能,但需根据患者情况进行个体化替代治疗,以便为患者提供安全有效的手术条件及术后恢复。

Objective To recognize and explore alternative therapy during perioperative period for coagulation factorⅪdeficiency complicated with surgical diseases,especially with giant tumors.Methods Through literature review and case summary,APTT,Ⅺconcentration and TEG was monitored before and after operation for 2 patients with coagulation factorⅪdeficiency,and fresh frozen plasma was injected and assisted with other treatments.Results After FFP transfusion,APTT was shortened from 103.6 s to 46.5-57.1 s and from 87.7 s to 42.8-46.6 s in coagulation factorⅪdeficiency patients complicated with giant tumor and appendicitis,respectively.For patients with tumor,operations were conducted when APTT was controlled at 46.5 s,with no massive intraoperative bleeding,and the postoperative APTT was tested between 46.5 and57.1 s,with no clinical evidence of bleeding or exudation.For patients with appendicitis,operations were conducted when APTT was controlled at 46.6 s,with a little intraoperative bleeding,and the postoperative APTT was tested between 42.8 and 46.6 s,with no clinical evidence of bleeding or exudation.Conclusion Peri-operative fresh frozen plasma transfusion for patients with coagulation factor XI deficiency complicated with surgical diseases can significantly improve the coagulation function of the patients,but individualized treatment should be carried out according to the patients’conditions aiming at providing safe and effective surgical conditions and postoperative recovery.