PB1224 - Bleeding symptoms and hemostatic alterations in Thai hemophilia carriers: A case-control study

Background: As hemophilia is a X-linked recessive disorder, male patients with hemophilia usually have bleeding symptoms, are diagnosed with the diseases and receive proper treatment. However, hemophilia carriers also at risk of bleeding tendency, however, they are usually overlooked due to the mode of inheritance.

Aims: This case-control study was conducted to compare the bleeding symptoms and hemostatic alterations among obligate hemophilia carriers, possible hemophilia carriers and age-matched female healthy control

Methods: All obligate and possible hemophilia carriers which were classified by history taking and female healthy controls were enrolled. They were asked to fill up the demographic data and Thai version of ISTH-BAT. Then, they were tested for CBC, PT/INR, APTT, FVIII:C, FIX:C, VWF:Ag and VWF:GPIbM. The data were analyzed and statistical significance was accepted when P < 0.05.

Results: Forty female hemophilia carriers (A 28, B 12) and 41 aged matched female healthy controls were enrolled. Fivteen were obligate carriers while 25 were possible carriers. The demographic and laboratory data were as shown in Table 1. The two common bleeding symptoms in hemophilia carriers were bleeding after dental procedures (23%) and heavy menstrual bleeding (18%) and post-partum bleeding (10%) while 48% had no bleeding symptoms. ISTH-BAT scores in obligate carriers were higher than possible carriers and controls (Table 2). Mean FVIII:C level in hemophilia A carriers and mean FIX:C level in hemophilia B carriers were significantly lower than controls (Table 2). Thirty-six percent of hemophilia A carriers and 25% of hemophilia B carriers should be diagnosed as female mild hemophilia as their factor clotting levels were lower than 40%.

Conclusion(s): Hemophilia carriers, especially obligate carriers, also have bleeding tendency as mild hemophilia in one-forth to one-third of hemophilia carriers. This issue should be more emphasized in hemophilia treatment center to provide proper advice to prevent serious bleeding in this specific group.