摘要
目的 探讨血友病A伴抑制物的免疫耐受诱导（immune tolerance induction,ITI）治疗,提高血友病A伴抑制物患者的诊疗水平.方法 对重型血友病A患者用APTT标准曲线一期法测定凝血因子Ⅷ（FⅧ）活性（FⅧ∶C）;用Bethesda法定量测定FⅧ抗体;用长距离PCR方法检测内含子22倒位.结果 经检测发现患者为FⅧ基因22内含子倒位;ITI治疗3个月后,患者FⅧ抑制物滴度下降为0,输注FⅧ后回收率＞66%,治疗6个月后达到抑制物清除标准,停止治疗.结论 该患者是国内首例采用ITI成功治疗血友病A伴抑制物的病例,ITI是目前最有希望根除血友病抑制物的方法.
Objective To explore the immune tolerance induction （ITI） in a severe hemophilia A sayed by one-stage method and FⅧ antibody by Bethesda method. Mutation screening of FⅧ gene intron 22 inversion was performed using LD-PCR. Results FⅧ gene intron 22 inversion was detected in this patient.Clinical tolerance to FⅧ was successfully induced after administration of the ITI regimen combined with immunosuppression. A fall of inhibitor titer from 8 BU to 0 BU after treatment for 3 months, and in vivo FⅧ recovery （ 〉 66% ） was normalized. The patient had no bleeding episode in the following 6 months. Conclusion This is the first case report on successful immune tolerance induction therapy in Chinese hemophilia A patient. ITI is the most effective therapy for hemophilia A with inhibitor.
出处
《中华血液学杂志》
CAS
CSCD
北大核心
2010年第9期577-580,共4页
Chinese Journal of Hematology
关键词
血友病A
抑制物
免疫耐受
诱导
Hemophilia A
Inhibitor
hnmune tolerance induction
