血友病A患者因子Ⅷ抑制物的检出率及抑制物产生的环境因素  

Frequency of Factor Ⅷ Inhibitor in the Patients with Hemophilia A and Environmental Risk factors for Inhibitor Development

作　　者：闫振宇[1] 范连凯[1] 李魁星[1] 王晓英[1] 华宝来[1] 王书杰[1] 赵永强[1] 

机构地区：[1]中国医学科学院北京协和医学院北京协和医院血液内科,北京100730

出　　处：《中国医学科学院学报》2009年第5期580-583,共4页Acta Academiae Medicinae Sinicae

基　　金：首都医学发展科研基金(2005-1024)~~

摘　　要：目的调查血友病A（HA）患者因子Ⅷ（FⅧ）抑制物的检出率,初步探讨抑制物产生的环境因素。方法以2003年4月-2007年4月在北京协和医院就诊的107例HA患者及在院外征集的158例HA患者（共265例）为研究对象,采用一期法检测FⅧ：C活性,Bethesda法检测FⅧ抑制物。结果265例HA患者中,22例（8.3%）抑制物检测为阳性,其中86.4%（19/22）为低反应者（抑制物滴度≤5 000 BU/L）,13.6%（3/22）为高反应者（抑制物滴度〉5 000 BU/L）。年龄〉50岁患者的抑制物检出率为50%,明显高于其他年龄组（P=0.000）。在158例临床治疗资料较完整的新征集患者中,FⅧ制剂输注频率大于12次/年者的抑制物阳性率为12.8%,而输注频率小于12次/年者为5.8%,两组相比差异无统计学意义（P=0.156）。有短时间内持续输注FⅧ史者的抑制物阳性率明显高于无持续输注FⅧ史的患者（28.5%vs.1.6%,P=0.000）。应用多种品牌和单一品牌FⅧ制剂患者的抑制物阳性率分别为9.3%和3.9%,两组相比差异无统计学意义（P=0.229）。结论HA患者抑制物的生成可能与患者年龄、短时间内持续输注FⅧ史等因素有关。Objective To screen for factor Ⅷ inhibitor in patients with hemophilia A （HA） and explore the environmental risk factors for inhibitor development. Methods Totally 265 patients with HA were enrolled, including 107 consecutive inpatients and outpatients in Peking Union Medical College Hospital from April 2003 to April 2007 and 158 patients newly recruited from other hospitals. FⅧ ： C activity was measured by one-stage coagulation assay. FⅧ inhibitor was determined using Bethesda method. Results In 265 HA patients, FⅧ inhibitor was detected in 22 patients （8.3%）. Nine of them （ 86.4% ） were low responders （ inhibitor titers ≤5 000 BU/L）, 3 （ 13.6% ） were high responders （ the titers 〉 5 000 BU/L）. The frequency of FⅧ inhibitor was 50% in the patients aged over 50 years, which was significantly higher than those in other age groups （ P =0. 000）. Among 158 newly recruited patients with full clinical data, the frequency of FⅧ inhibitor was 12.8% in patients who had received infusion of FⅧ products for more than 12 doses on average each year, while it was 5, 8% in whom the infusion doses were less than 12 （P =0. 156）. The frequency of FⅧ inhibitor was 28.5% in patients with a history of continuous infusion of FⅧ products whereas it was only 1.6% in patients without such history （ P = 0. 000）. In patients who exposed to multiple-branded or single- branded FⅧ products, the frequencies of FⅧ inhibitor were 9.3% and 3.9%, respectively （P = 0. 229）. Conclusion The development of factor Ⅷ inhibitor in patients with hemophilia A may be related to the age and the history of continuous infusion of FⅧ products.

关 键 词：血友病A 因子Ⅷ 抑制物 环境因素 

分 类 号：R554.1[医药卫生—血液循环系统疾病]