中国当代儿科杂志2025，Vol.27Issue(3)：293-299,7.DOI:10.7499/j.issn.1008-8830.2409099
血栓弹力图评估艾美赛珠单抗治疗后重型血友病A患儿凝血功能的单中心研究
Application value of thromboelastography in assessing coagulation function in children with severe hemophilia A after emicizumab therapy:a single-center study
摘要
Abstract
Objective To investigate the application value of thromboelastography(TEG)in assessing coagulation function in children with severe hemophilia A(HA)after emicizumab(EMI)therapy.Methods A retrospective analysis was performed on the activated partial thromboplastin time(APTT)and TEG testing results of 17 children with severe HA before and after EMI treatment at Shenzhen Children's Hospital from January 2023 to July 2024.Correlation analysis was conducted between coagulation factor VIII(FVIII)equivalent activity and reaction time(R value)measured by TEG.Results After EMI treatment,the mean bleeding rate for children with severe HA was 1.6 events per year,with 15 children(88%)without spontaneous bleeding or joint bleeding.The children with severe HA showed a significant reduction in APTT after EMI treatment(P<0.05),with a significantly shorter APTT than the normal control group(P<0.05).There was no correlation between APTT and FVIII equivalent activity after treatment(P>0.05).After EMI treatment,TEG parameters,including R value,kinetic time,alpha angle(α),maximum amplitude,clot strength,and coagulation index,shifted from a hypocoagulable state before treatment to a nearly normal state after treatment(P<0.05).The R value demonstrated a strong negative correlation with FVIII equivalent activity(r=-0.758,P<0.05).Conclusions The bleeding condition of children with severe HA can be effectively controlled after EMI treatment.Routine APTT testing cannot reflect true coagulation function,whereas TEG testing is clinically valuable in assessing the coagulation function of children with severe HA undergoing EMI treatment.关键词
血友病A/艾美赛珠单抗/血栓弹力图/实验室检测/儿童Key words
Hemophilia A/Emicizumab/Thromboelastography/Laboratory testing/Child引用本文复制引用
彭冬,王缨,周桂池,李倩,罗美珠,罗丽萍,邝雅贤,付笑迎..血栓弹力图评估艾美赛珠单抗治疗后重型血友病A患儿凝血功能的单中心研究[J].中国当代儿科杂志,2025,27(3):293-299,7.基金项目
广东省高水平医院建设专项基金 （）
深圳市医疗卫生"三名工程"(SZSM202211033). （SZSM202211033）
