Clotting factor level is not a good predictor of bleeding in carriers of haemophilia A and B
Clotting factor level is not a good predictor of bleeding in carriers of haemophilia A and B
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DOI：
10.1097/mbc.0000000000000083
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发表时间：
2014-07-01
影响因子：
1.1
通讯作者：
Baghaei, Fariba
中科院分区：
文献类型：
--
作者：
Olsson, Anna;Hellgren, Margareta;Baghaei, Fariba
已知血友病携带者具有广泛的凝血因子水平和出血症状。本研究旨在调查重度和中度血友病携带者与对照组相比是否有增加的出血倾向，使用由1型VWD诊断和管理分子和临床标志物研究组（MCMDM-1VWD）开发的出血评估工具的浓缩版本。126名经基因验证的重度和中度血友病携带者和90名对照者就出血症状进行了访谈。出血评分至少为4分即为阳性，表明有明显出血倾向。检测携带者的凝血因子水平。B型血友病携带者19例，平均因子(F)IX:C水平为0.54(+/- 0.27)kIU/l； a型血友病携带者107例，平均因子(F)IX:C水平为0.74 (+/- 0.32)kIU/l。携带者中位出血评分为2(-3-12)，对照组中位出血评分为-1 （-3-8）
Carriers of haemophilia are known to have a wide range of clotting factor levels and bleeding symptoms. This study aimed at investigating whether carriers of severe and moderate haemophilia had an increased bleeding tendency, compared with a control group, using a condensed version of a bleeding assessment tool developed by the Molecular and Clinical Markers for the Diagnosis and Management of Type 1 VWD study group (MCMDM-1VWD). One hundred and twenty-six genetically verified carriers of severe and moderate haemophilia and 90 controls were interviewed regarding bleeding symptoms. A bleeding score of at least 4 was considered positive, indicating a significant bleeding tendency. Clotting factor levels were tested in the carriers. Nineteen of the women were carriers of haemophilia B, with a mean factor (F)IX:C level of 0.54(+/- 0.27) kIU/l, and 107 were carriers of haemophilia A, with a mean FVIII: C level of 0.74 (+/- 0.32) kIU/l. The median bleeding score was 2 (-3-12) among carriers and -1 (-3-8) among controls (P