凝血因子Ⅺ缺乏症患者2次手术出血死亡原因思考

Fatal case analysis of a factor Ⅺ deficient patient died of second surgery bleeding

目的分析本例凝血因子Ⅺ（FⅪ）缺乏症2次手术出血死亡的原因,为该疾病的及时诊断与有效治疗提供参考。方法回顾性查阅本病例相关病程记录、输血资料、实验室检查及相关文献报道,分析患者死亡原因。结果患者在手术前未发现凝血功能障碍,手术过程顺利,术后手术部位大量出血,行2次开胸探查止血手术,疑似凝血功能障碍,经过大量输血等抢救措施,患者最终抢救无效死亡。结合实验室凝血结果、TEG和凝血因子检查,明确患者为FⅪ缺乏症。结论 FⅪ缺乏症合并外科疾病需手术治疗时,术前应进行详细的检查评估和多学科联合会诊,制定详尽的输血方案,避免此类事件发生。

Objective To analyze the reason for the death of a factor XI deficient patient caused by second surgery bleeding and to provide reference data for the diagnosis and treatment strategy of factor XI deficiency. Methods Clinical records, transfusion data, and laboratory data were studied retrospectively. Relevant literatures were reviewed. Results Blood coagulation dysfunction was not discovered pre-operation. The operation was successfully performed. However, severe postoperative bleeding occurred. Chest hemostasis operation was performed. The patient was suspected of blood coagulation dysfunction. Despite various emergency therapies applied, the patient died eventually. According to laboratory coagulation tests, TEG and blood coagulation factor detection, the patient was diagnosed with factor Ⅺ deficiency. Conclusion As for F Ⅺ deficient patients, muhidisciplinary joint consultation is required before any operations. Detailed inspection and evaluation need to be made before the operation. A detailed blood transfusion therapy need to be planned in advance to avoid potential fatality.