학술논문
超小剂量FⅧ诱导血友病A伴FⅧ抑制物患者免疫耐受8例临床观察 / Immune tolerance induction therapy for 8 patients with hemophilia A and FⅧ inhibitor using ultra-low-dose regimens
Document Type
Academic Journal
Author
范憬超; 蔡力生; 王丽丽; 周昭贵; 刘泽林; 王缨; 蔡云; 杨仁池; 杜新; Fan Jingchao; Cai Lisheng; Wang Lili; Zhou Zhaogui; Liu Zelin; Wang Ying; Cai Yun; Yang Renchi; Du Xin
Source
血栓与止血学 / Chinese Journal of Thrombosis and Hemostasis. 29(6):260-265
Subject
Language
Chinese
ISSN
1009-6213
Abstract
目的 探讨血友病A伴抑制物患者免疫耐受诱导(ITI)的方法,尝试寻找适合我国患者的治疗方案.方法 对中型/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;抑制物滴度>0.6 BU/mL的患者停用FⅧ制剂,待FⅧ抑制物阴性(<0.6 BU/mL)后用超小剂量FⅧ(初始剂量1～2.5 IU·kg-1·d-1)联合地塞米松±利妥昔单抗进行ITI治疗.结果 8例伴FⅧ抑制物患者,3例患者获得完全耐受,2例患者达到部分耐受,3例诱导失败患者转为传统剂量FⅧ(50 IU/kg,隔天1次)ITI治疗,获得完全耐受.结论 使用超小剂量FⅧ进行ITI治疗的有效率可达62.5%,该治疗方案具有一定的社会及经济价值.
Objective To explore a method of inducing immune tolerance in patients with hemophilia A and FⅧ inhibitor suitable for Chinese patients.Methods The FⅧ:C was assayed by one-stage method and FⅧ antibody by Bethesda method.Once the inhibitors of HA patients were turned into negative after the discontinuation of FⅧ,patients were treated by ITI using ultra-low-dose regimens(starting with 1～2.5 IU·Kg-1·d-1)combined with dexamethasone±rituximab.Results Complete tolerance was achieved in 3 patients,and 2 patients got partial tolerance.The other 3 patients had no response to ITI with ultra-low-dose regimen,however,after they were transferred to high-dose ITI regimen and achieved complete tolerance.Conclusion ITI using ultra-low-dose ITI,whose response rate reached 62.5％,could be of certain social and economic significance.
Objective To explore a method of inducing immune tolerance in patients with hemophilia A and FⅧ inhibitor suitable for Chinese patients.Methods The FⅧ:C was assayed by one-stage method and FⅧ antibody by Bethesda method.Once the inhibitors of HA patients were turned into negative after the discontinuation of FⅧ,patients were treated by ITI using ultra-low-dose regimens(starting with 1～2.5 IU·Kg-1·d-1)combined with dexamethasone±rituximab.Results Complete tolerance was achieved in 3 patients,and 2 patients got partial tolerance.The other 3 patients had no response to ITI with ultra-low-dose regimen,however,after they were transferred to high-dose ITI regimen and achieved complete tolerance.Conclusion ITI using ultra-low-dose ITI,whose response rate reached 62.5％,could be of certain social and economic significance.