CAJ | 학술논문

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≤5000 BU/L),13.6%(3/22)为高反应者(抑制物滴度>5000 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5.89b,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.
목적 조사혈우병A(HA)환자인자Ⅷ(FⅧ)억제물적검출솔,초보탐토억제물산생적배경인소.방법 이2003년4월～2007년4월재북경협화의원취진적107례HA환자급재원외정집적158례HA환자(공265례)위연구대상,채용일기법검측FⅧ:C활성,Bethesda법검측FⅧ억제물.결과 265례HA환자중,22례(8.3%)억제물검측위양성,기중86.4%(19/22)위저반응자(억제물적도≤5000 BU/L),13.6%(3/22)위고반응자(억제물적도>5000 BU/L).년령>50세환자적억제물검출솔위50%,명현고우기타년령조(P=0.000).재158례림상치료자료교완정적신정집환자중,FⅧ제제수주빈솔대우12차/년자적억제물양성솔위12.8%,이수주빈솔소우12차/년자위5.89b,량조상비차이무통계학의의(P=0.156).유단시간내지속수주FⅧ사자적억제물양성솔명현고우무지속수주FⅧ사적환자(28.5% vs.1.6%,P=0.000).응용다충품패화단일품패FⅧ제제환자적억제물양성솔분별위9.3%화3.9%,량조상비차이무통계학의의(P=0.229).결론 HA환자억제물적생성가능여환자년령、단시간내지속수주FⅧ사등인소유관.
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.