Bleeding in carriers of hemophilia
Bleeding in carriers of hemophilia
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DOI：
10.1182/blood-2005-09-3879
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发表时间：
2006-07-01
期刊：
影响因子：
20.3
通讯作者：
Rosendaal, Frits R.
中科院分区：
文献类型：
--
作者：
Plug, Iris;Mauser-Bunschoten, Eveline P.;Rosendaal, Frits R.
在血友病杂合子携带者和非携带者中，都观察到了广泛的第VIII和IX因子水平。在女性携带者中，极端的裂解可能会导致凝血因子水平低下。我们研究了杂合子血友病携带者对出血症状发生的影响。2001年前在荷兰接受血友病携带者检测的大多数妇女都进行了邮寄调查。问卷内容包括患者的个人特征、家庭成员的血友病特征、携带者检测以及拔牙后出血、扁桃体摘除术后出血等出血史。有关凝血因子水平的信息从医院图表中获得。用Logistic回归分析携带者状态和凝血因子水平与出血事件发生的关系。2004年，发出了766份调查问卷，546名妇女(80%)作出了答复。的。血友病A、B携带者274例。携带者凝血因子水平中位数为0.60IU/mL(0.05~2.19IU/mL)，非携带者为1.02IU/mL(0.45~3.28IU/mL)。凝血因子水平从0.60到0.05IU/ml与小伤口出血时间延长和拔牙、扁桃体切除和手术后出血时间延长的关系日益密切。血友病携带者比其他女性出血更多，特别是在药物干预后。我们的发现表明，不仅凝血因子水平在分布的极端，类似于轻度血友病，而且凝血因子水平在0.41至0.60IU/毫升之间轻度降低与出血有关。
A wide range of factor VIII and IX levels is observed in heterozygous carriers of hemophilia as well as in noncarriers. In female carriers, extreme lyonization may lead to low clotting factor levels. We studied the effect of heterozygous hemophilia carriership on the occurrence of bleeding symptoms. A postal survey was performed among most of the women who were tested for carriership of hemophilia in the Netherlands before 2001. The questionnaire included items on personal characteristics, characteristics of hemophilia in the affected family members, and carrier testing and history of bleeding problems such as bleeding after tooth extraction, bleeding after tonsillectomy, and other operations. Information on clotting factor levels was obtained from the hospital charts. Logistic regression was used to assess the relation of carrier status and clotting factor levels with the occurrence of hemorrhagic events. In 2004, 766 questionnaires were sent, and 546 women responded (80%). Of. these, 274 were carriers of hemophilia A or B. The median clotting factor level of carriers was 0.60 IU/mL (range, 0.05-2.19 IU/mL) compared with 1.02 IU/mL (range, 0.45-3.28 IU/mL) in noncarriers. Clotting factor levels from 0.60 to 0.05 IU/mL were increasingly associated with prolonged bleeding from small wounds and prolonged bleeding after tooth extraction, tonsillectomy, and operations. Carriers of hemophilia bleed more than other women, especially after medical interventions. Our findings suggest that not only clotting factor levels at the extreme of the distribution, resembling mild hemophilia, but also mildly reduced clotting factor levels between 0.41 and 0.60 IU/mL are associated with bleeding.