凝血因子Ⅴ缺乏症患者的围手术期血液管理并文献复习  

Perioperative blood management of a patient with coagulation factorⅤdeficiency and literature review

作　　者：史荣辉 孟强 吴建君 杨兰 龙小琼 张丽 赵树铭 SHI Ronghui;MENG Qiang;WU Jianjun;YANG Lan;LONG Xiaoqiong;ZHANG Li;ZHAO Shuming(Department of Blood Transfusion,Guiqian International General Hospital,Guiyang 550018,China;Department of Clinical Laboratory,Guiqian International General Hospital,Guiyang 550018,China)

机构地区：[1]贵黔国际总医院输血科,贵阳贵州550018 [2]贵黔国际总医院检验科,贵阳贵州550018

出　　处：《中国输血杂志》2023年第11期1057-1060,共4页Chinese Journal of Blood Transfusion

摘　　要：目的探讨胰腺假性囊肿合并凝血因子Ⅴ(FⅤ)缺乏症患者的围手术期血液管理。方法术前:为判明冷沉淀和新鲜冰冻血浆(FFP)对提升FⅤ的效果,采用在静息状态下,交替输注冷沉淀和FFP,监测输注前、输注后1 h、24 h和48 h TEG、凝血功能及凝血因子活性,制定术中及术后输血策略,术前补充FFP 600 mL和冷沉淀10 U。术中:手术进行7 h,输注FFP 600 mL,无明显出血。术后:输注FFP。结果术前:患者凝血FⅤ活性2次检测结果分别为1.9%和1.8%,交替输注冷沉淀10 U和FFP 1200 mL后,FⅤ活性均升高,分别为5.1%和6.0%;TEG检测各指标无明显差异,PT和ATPP有不同程度降低。术中:手术顺利,无明显出血。术后:术后2 h输注FFP 500 mL,术后1—7 d每天输注FFP 250~500 mL,患者伤口未见明显渗血,TEG、PT、APTT和血红蛋白(Hb)与术前比较变化不大,患者于术后12 d顺利出院。基因检测结果为遗传性凝血FⅤ缺乏症,属于复合杂合变异。结论FⅤ缺乏症合并外科疾病患者的围手术期血液管理,需结合实验室检查和临床表现进行输血前评估和输血后评价,冷沉淀和FFP均可以有效补充凝血因子。判断FⅤ缺乏症患者的凝血状态时,TEG检测似乎优于凝血功能7项检测。Objective To explore the perioperative blood management in patients with pancreatic pseudocyst combiend with coagulation factor Ⅴ(FⅤ)deficiency.Methods Preoperative:In order to determine the effect of cryoprecipitated anti-hemophilic factor and fresh frozen plasma(FFP)on the elevation level of factor Ⅴ,we alternately infused cryoprecipitate and FFP in the resting state.TEG,coagulation function and coagulation factor activity were monitored before and 1 h,24 h and 48 h after infusion,and intraoperative and postoperative blood transfusion strategies were formulated.FFP 600 mL and cryoprecipitate 10 U were supplemented preoperatively.Intraoperative:The operation procedure was performed for 7 hours with an infusion of 600 mL FFP without significant bleeding.Postoperative:FFP was infused.Results Preoperative:The coagulation factor Ⅴ activity on pre-operation was 1.9% and 1.8%.After alternating infusion cryoprecipitate 10 U and FFP 1200 mL,the FⅤ activity increased to 5.1% and 6.0%,respectively.There was no significant difference in TEG parameters,PT and ATPP results were decreased to varying degrees.Intra-operative:The operation was successful without obvious bleeding.Postoperative:FFP 500 mL was infused 2 h after operation,and FFP 250-500 mL was injected daily from 1 to 7 days after surgery.No significant bleeding was observed in the wound,the results of TEG,PT,APTT and hemoglobin(Hb)did not change significantly compared with those before surgery.The patient was discharged successfully 12 days after surgery.The genetic test results showed that he had inherited coagulation factor Ⅴ deficiency,which was a compound heterozygous variation.Conclusion Perioperative blood management in patients with FⅤ deficiency combined with surgical disease,requiring pre-transfusion evaluation and post-transfusion evaluation in combination with laboratory investigations and clinical manifestations,cryoprecipitate and fresh frozen plasma can be effective in supplementing coagulation factors.The TEG seems to be better than th

关 键 词：凝血因子Ⅴ缺乏 冷沉淀 新鲜冰冻血浆(FFP) 血栓弹力图(TEG) 

分 类 号：R457.1[医药卫生—治疗学]