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[甲型血友病患者中因子VIII抑制剂的发生率及抑制剂产生的环境危险因素]

[Frequency of factor VIII inhibitor in the patients with hemophilia A and environmental risk factors for inhibitor development].

作者信息

Yan Zhen-Yu, Fan Lian-Kai, Li Kui-Xing, Wang Xiao-Ying, Hua Bao-Lai, Wang Shu-Jie, Zhao Yong-Qiang

机构信息

Department of Hematology, PUMC Hospital, CAMS and PUMC, Beijing 100730, China.

出版信息

Zhongguo Yi Xue Ke Xue Yuan Xue Bao. 2009 Oct;31(5):580-3.

PMID:19968075
Abstract

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 < or = 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.

摘要

目的

筛查甲型血友病(HA)患者中的凝血因子VIII抑制物,并探讨抑制物产生的环境危险因素。

方法

共纳入265例HA患者,其中包括2003年4月至2007年4月在北京协和医院连续收治的107例住院及门诊患者,以及从其他医院新招募的158例患者。采用一期凝血法检测FVIII:C活性。使用贝塞斯达法测定FVIII抑制物。

结果

265例HA患者中,检测到22例(8.3%)存在FVIII抑制物。其中9例(86.4%)为低反应者(抑制物滴度≤5000 BU/L),3例(13.6%)为高反应者(滴度>5000 BU/L)。50岁以上患者中FVIII抑制物的发生率为50%,显著高于其他年龄组(P = 0.000)。在158例有完整临床资料的新招募患者中,平均每年接受FVIII制品输注超过12剂的患者中FVIII抑制物的发生率为12.8%,而输注剂量少于12剂的患者中该发生率为5.8%(P = 0.156)。有连续输注FVIII制品史的患者中FVIII抑制物的发生率为28.5%,而无此病史的患者中仅为1.6%(P = 0.000)。在接触多品牌或单品牌FVIII制品的患者中,FVIII抑制物的发生率分别为9.3%和3.9%(P = 0.229)。

结论

甲型血友病患者中凝血因子VIII抑制物的产生可能与年龄及连续输注FVIII制品的病史有关。

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Zhongguo Yi Xue Ke Xue Yuan Xue Bao. 2009 Oct;31(5):580-3.
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