The management of menorrhagia presents a challenge in women with severe bleeding disorders. Conservative medical management is the first line treatment and most women with severe bleeding disorder require combination treatment. Surgical intervention may ultimately be offered to women in whom medical management has failed and whom no longer desire fertility. Women with low factor levels are at risk of perioperative bleeding complications and may require haemostatic support. A total of 50 women with severe factor deficiencies (less than 20iu/dL) were included in this study. 46 women were registered at the Haemophilia Centre at the Royal Free Hospital in London. Four cases were also included from the Rhine-Ruhr Haemophilia Centre in Duisburg, Germany. We reviewed the occurrence of menorrhagia and the management options that were offered. In those that required surgical intervention, the incidence of postoperative bleeding complications and the requirement for factor concentration was also reviewed.
The bleeding disorders in these women were 34 (68%) with severe factor XI deficiency, 10 (20%) with severe type 1 and type 3 von Willebrand's disease, 4 (8%) with factor VII deficiency, 2 (4%) had factor V or X deficiencies and one (2%) had a combination of factor VI and VIII deficiency. The ISTH/SSC joint working group bleeding assessment tool was used to assess the severity and frequency of bleeding symptoms among this cohort of women. The bleeding scores ranged from −2 to 30 with a median score of 9.5.
In total, 32 out of 50 (64%) women with severe factor deficiency required medical attention for menorrhagia. Medical treatment included hormonal preparations (combined oral contraceptive pill or levonorgestrel intrauterine device), which was used as a first line treatment in 15 out of 32 (46.8%) women. Haemostatic treatment included antifibrinolytic medication such as tranexamic acid, which was used in combination with hormonal therapy. One women required intranasal DDAVP, von Willebrand factor concentrate and tranexamic acid. Failure to control menstrual bleeding occurred in 14 (43.7%) women and surgical intervention was required. 7 out of 14 (50%) women required hysterectomy and the remaining 7 women underwent endometrial ablation. Prophylaxis with factor concentration to cover surgical intervention was given in 8 out of 14 women (64.2%). The remainder received tranexamic acid for 24–48 hours following surgery. Postoperative bleeding occurred in 7 women that had surgical intervention, despite two women receiving prophylaxis. This study highlights the complexity involved in the management of menorrhagia in women with severe bleeding disorders and the high risk of postoperative bleeding.
No relevant conflicts of interest to declare.
Asterisk with author names denotes non-ASH members.