血友病A患者关节置换术围手术期使用改良凝血因子Ⅷ替代方案的疗效观察

Therapeutic Effect of Modified Coagulation Factor Ⅷ Substitution Used During Perioperative Period of Total Joint Replacement in Patients with Hemophilia A

目的 探讨改良凝血因子Ⅷ(FⅧ)替代方案对血友病A患者关节置换术(TJR)疗效的影响.方法 回顾性分析2010年1月—2016年12月收治的7例血友病A患者资料,其中4例行全髋关节置换术(THA),3例行全膝关节置换术(TKA).分别评价各患者围手术期FⅧ用量、FⅧ水平、悬浮红细胞输血量、全血丢失量(TBL)、显性失血量(DBL)、隐性失血量(HBL)、THA前后患者Harris髋关节评分(HHS)、TKA前后患者膝关节评分(KSS)及术后并发症.结果 FⅧ的总用量中位数为458.5 IU/kg(范围:290.0~792.6 IU/kg),术后第2、5、8天中位FⅧ水平分别为82.0%、70.0%、32.0%.悬浮红细胞输注量中位数为2 U(范围:0~5 U).THA患者中位TBL、DBL、HBL分别为1452 mL(范围:750~2568 mL)、625 mL(范围:365~800 mL)、937 mL(范围:132~1768 mL),TKA患者中位TBL、DBL、HBL分别为1082 mL(范围:541~1732 mL)、437 mL(范围:244~665 mL)、687 mL(范围:337~956 mL).THA患者中位HHS评分由术前37分(范围:30~43分)改善至术后84分(范围:80~90分),TKA患者中位KSS评分由27分(范围:20~33分)改善至93分(范围:90~95分).除1例患者术后左膝出现血肿外,其余患者在围手术期及术后随访期均未出现并发症.结论 通过对血友病A患者围手术期FⅧ用量、FⅧ水平、失血量、悬浮红细胞输血量、术后关节功能改善及并发症的发生情况进行研究,证实了改良FⅧ替代方案用于血友病A患者关节置换术是安全和有效的.

Objective To retrospectively evaluate the efficacy of a modified factor Ⅷ(FⅧ)substitution therapy for total joint replacement(TJR)in patients with hemophilia A.Methods 7 patients with hemophilia A were retrospectively analyzed including 4 patients undergoing total hip arthroplasty(THA)and 3 undergoing total knee arthroplasty(TKA).Perioperative FVIH consumption,FVB level,total blood loss(TBL),dominant blood loss(DBL)and hidden blood loss(HBL),red blood cell(RBC)transfusion,Harris hip score(HHS)of patients before and after THA,Knee society score(KSS)of patients before and after TKA and postoperative complications were individually assessed.Results The total consumption of FⅧ was 458.5 IU/kg(range:290.0-792.6 IU/kg)in median.The median FVIII level was 82.0%,70.0%and 32.0%at postoperative day 2,5 and 8.The median total RBC transfusion was 2 U(range:0-5 U).The median amount of TBL,DBL and HBL were 1452 mL(range:750-2568 mL),625 mL(range:365・800 mL)and 937 mL(range:132-1768 mL)for patients undergoing THA,and 1082 mL(range:541-1732 mL),437 mL(range:244-665 mL)and 687 mL(range:337-956 mL)for patients undergoing TKA.The median HHS improved from 37(range:30-43)to 84(range:80-90),and the KSS improved from 27(range:20-33)to 93(range:90-95)before surgery and at the last follow-up.Except for 1 patient who had a hematoma in the left knee after surgery,the other patients had no complications during the perioperative period and the postoperative follow-up period.Conclusion The modified FV1H substitution strategy for TJR in patients with hemophilia A is safe and effective demonstrated by satisfied results regarding the consumption of FVHI,FVHI level,blood loss,RBC transfusion joint function improvement and complication.