Paroskie Allison, Gailani Dave, DeBaun Michael R, Sidonio Robert F
Vanderbilt University, Nashville, TN, USA.
Monroe Carell Jr. Children's Hospital at Vanderbilt, Nashville, TN, USA.
Br J Haematol. 2015 Jul;170(2):223-8. doi: 10.1111/bjh.13423. Epub 2015 Apr 1.
Haemophilia A carriers have historically been thought to exhibit normal haemostasis. However, recent data demonstrates that, despite normal factor VIII (FVIII), haemophilia A carriers demonstrate an increased bleeding tendency. We tested the hypothesis that obligate haemophilia carriers exhibit an increase in clinically relevant bleeding. A cross-sectional study was performed comparing haemophilia A carriers to normal women. Questionnaire assessment included a general bleeding questionnaire, condensed MCMDM-1VWD bleeding assessment tool and Pictorial Bleeding Assessment Chart (PBAC). Laboratory assessment included complete blood count, prothrombin time, activated partial thromboplastin time, fibrinogen activity, FVIII activity (
C), von Willebrand factor antigen level, ristocetin cofactor, platelet function analyser-100(TM) and ABO blood type. Forty-four haemophilia A carriers and 43 controls were included. Demographic features were similar. Laboratory results demonstrated a statistically significant difference only in
C (82·5 vs. 134%, P < 0·001). Carriers reported a higher number of bleeding events, and both condensed MCMDM-1 VWD bleeding scores (5 vs. 1, P < 0·001) and PBAC scores (423 vs. 182·5, P = 0·018) were significantly higher in carriers. Haemophilia A carriers exhibit increased bleeding symptoms when compared to normal women. Further studies are necessary to fully understand the bleeding phenotype in this population and optimize clinical management.
历史上一直认为甲型血友病携带者的止血功能正常。然而，最近的数据表明，尽管因子VIII（FVIII）水平正常，但甲型血友病携带者的出血倾向增加。我们检验了一个假设，即甲型血友病携带者在临床上相关的出血情况会增加。进行了一项横断面研究，将甲型血友病携带者与正常女性进行比较。问卷调查评估包括一份一般出血问卷、简化的MCMDM-1VWD出血评估工具和图像出血评估表（PBAC）。实验室评估包括全血细胞计数、凝血酶原时间、活化部分凝血活酶时间、纤维蛋白原活性、FVIII活性（FVIII：C）、血管性血友病因子抗原水平、瑞斯托霉素辅因子、血小板功能分析仪-100（TM）和ABO血型。纳入了44名甲型血友病携带者和43名对照者。人口统计学特征相似。实验室结果仅在FVIII：C方面显示出统计学上的显著差异（82.5%对134%，P<0.001）。携带者报告的出血事件数量更多，并且携带者的简化MCMDM-1VWD出血评分（5对1，P<0.001）和PBAC评分（423对182.5，P=0.018）均显著更高。与正常女性相比，甲型血友病携带者表现出更多的出血症状。需要进一步研究以充分了解该人群的出血表型并优化临床管理。
