BACKGROUND Factor XI(FXI)deficiency,also known as hemophilia C,is a rare bleeding disorder of unpredictable severity that correlates poorly with FXI coagulation activity.This often poses great challenges in perioperat...BACKGROUND Factor XI(FXI)deficiency,also known as hemophilia C,is a rare bleeding disorder of unpredictable severity that correlates poorly with FXI coagulation activity.This often poses great challenges in perioperative hemostatic management.Thromboelastography(TEG)is a method for testing blood coagulation using a viscoelastic hemostatic assay of whole blood to assess the overall coagulation status.Here,we present the successful application of intraoperative TEG monitoring in an FXI-deficient patient as an individualized blood transfusion strategy.CASE SUMMARY A 21-year-old male patient with FXI deficiency was scheduled to undergo reconstructive surgery for macrodactyly of the left foot under general anesthesia.To minimize his bleeding risk,he was scheduled to receive fresh frozen plasma(FFP)as an empirical prophylactic FXI replacement at a dose of 15-20 mL/kg body weight(900-1200 mL)before surgery.Subsequent FFP transfusion was to be adjusted according to surgical need.Instead,TEG assessment was used at the beginning and toward the end of his surgery.According to intraoperative TEG results,the normalization of coagulation function was achieved with an infusion of only 800 mL FFP,and blood loss was minimal.The patient showed an uneventful postoperative course and was discharged on postoperative day 8.CONCLUSION TEG can be readily applied in the intraoperative period to individualize transfusion needs in patients with rare inherited coagulopathy.展开更多
基金Education Reform Project Foundation for the Central Universities of Peking Union Medical College,No.2020zlgc0105Training Programme Foundation for Excellent Talent in Dongcheng District of Beijing,No.2019DCTM-08Non-profit Central Research Institute Fund of Chinese Academy of Medical Sciences,No.2019XK320018.
文摘BACKGROUND Factor XI(FXI)deficiency,also known as hemophilia C,is a rare bleeding disorder of unpredictable severity that correlates poorly with FXI coagulation activity.This often poses great challenges in perioperative hemostatic management.Thromboelastography(TEG)is a method for testing blood coagulation using a viscoelastic hemostatic assay of whole blood to assess the overall coagulation status.Here,we present the successful application of intraoperative TEG monitoring in an FXI-deficient patient as an individualized blood transfusion strategy.CASE SUMMARY A 21-year-old male patient with FXI deficiency was scheduled to undergo reconstructive surgery for macrodactyly of the left foot under general anesthesia.To minimize his bleeding risk,he was scheduled to receive fresh frozen plasma(FFP)as an empirical prophylactic FXI replacement at a dose of 15-20 mL/kg body weight(900-1200 mL)before surgery.Subsequent FFP transfusion was to be adjusted according to surgical need.Instead,TEG assessment was used at the beginning and toward the end of his surgery.According to intraoperative TEG results,the normalization of coagulation function was achieved with an infusion of only 800 mL FFP,and blood loss was minimal.The patient showed an uneventful postoperative course and was discharged on postoperative day 8.CONCLUSION TEG can be readily applied in the intraoperative period to individualize transfusion needs in patients with rare inherited coagulopathy.
