ميزان فاكتور هشت و فون ويلبراند و ارتباط آن‌ها با علايم باليني در ناقلين قطعي هموفيلي در اصفهان

The correlation of factor VIII and von Willebrand levels with clinical symptoms in obligate carriers of hemophilia A in Isfahan

Abstract Background and Objectives Bleeding tendency in obligate carriers of hemophilia A has been found since several years ago; some of haemophiliacs with factor levels of 40-60% also have abnormal bleeding. The present study is to measure factor VIII and von Willebrand levels and evaluate their correlation with clinical finding in obligate carriers. Materials and Methods In this cross-sectional study conducted 49 obligate carriers of Haemophilia A. F.VIII, vWF: Ag, PT, and APTT levels were checked and their correlationship with the bleeding score was evaluated and analyzed. Results Fourty-Nine Obligate carriers included in the study. The mean of PT, PTT , F.VIII, vWF: Ag and bleeding score (BS) were 12.6 ± 1.3, 36.3 ± 4.2, 52.5 ± 22.6, 83.7 ± 35.2, and 1.5 ± 1.7, respectively. The present study showed only 4% (2) of the carriers having had bleeding score (BS) equal to 5 and higher. Only F.VIII / vWF: Ag positively correlated with BS significantly (p = 0.006). Conclusions The present study showed that PT, PTT, and F.VIII levels and the bleeding score alone could not be helpful in screening obligate carriers. It seems that only determination of F.VIII/vWF: Ag ratio be helpful hn carrier states. But definitive diagnosis carrier states is by molecular genetic testing.