Assessment of menstrual blood loss and gynaecological problems in patients with inherited bleeding disorders

Abstract

Menstrual blood loss and gynaecological problems in patients with inherited bleeding disorders were assessed in this study. One hundred and sixteen women, including 66 with von Willebrand's disease (vWD), 30 carriers of haemophilia and 20 with factor XI (FXI) deficiency were interviewed and their gynaecological history obtained. Their case records were also reviewed and menstrual loss was objectively assessed using a pictorial blood assessment chart (PBAC). Comparison with an age-matched control group (69 women) was performed. Menorrhagia (PBAC score> 100) was confirmed in 74%, 57% and 59% of women with vWD, carriers of haemophilia and FXI deficiency, respectively, in comparison with 29% in the control group (P = 0.001). PBAC scores were higher in vWD patients with a von Willebrand factor activity (vWF:Ac) of </= 30 IU dL-1 compared to those with higher levels, but the difference was not statistically significant. However, there was no relation between PBAC score and the severity of the disease in FXI deficient patients and carriers of haemophilia. Duration of menstruation was significantly longer (P = 0.001) and episodes of flooding was significantly more common (P = 0.001) in patients with inherited bleeding disorders compared to the control group. However, there was no difference in the passage of clots during menstruation. Forty-seven per cent of patients with inherited bleeding disorders had consultations with their family practitioner or gynaecologist for menorrhagia, 36% had medical treatment and 27% had surgical procedures, including 10 hysterectomies. Post-operative bleeding followed in four out of 28 cases of hysteroscopy and/or dilatation and curettage. Bleeding complications following hysterectomy were reported in five out of 10 patients. In conclusion, menorrhagia is a common and major problem in patients with inherited bleeding disorders, especially vWD. Increased awareness among gynaecologists and haematologists of the high prevalence of menorrhagia and the treatment options available is necessary for optimal management of these patients. Appropriate preoperative assessment and haemostatic control during any gynaecological procedure, however minor, and in collaboration with the local haemophilia centre is essential to minimize risks of haemorrhagic complications.

von Willebrand's disease and menorrhagia: Prevalence, diagnosis, and management

“…Severe menorrhagia resulting in anemia or requiring transfusion was reported by 48-64% and 7-22% of women with type 1 vWD, respectively [18,24,25]. Women with vWD reported a significantly longer duration of bleeding and used a greater number of pads or tampons per cycle, compared to controls [18,25].…”

“…The reason for the racial difference is unclear because population screening suggests a similar prevalence of vWD among different racial groups [1]. The observation that African Americans have significantly higher vWF levels than whites could account for a lower frequency of the disorder [17,18]. However, the racial difference in prevalence persisted when a raceadjusted reference range was used for diagnosis [17].…”

“…In one study of women with objectively confirmed menorrhagia, 65% of women with vWD reported an onset at menarche compared to only 9% of those without a bleeding disorder [12]. Several other series found that menorrhagia began at menarche in the majority of women with vWD [18,24]. Affected women are also more likely to have a history of post-partum or post-operative bleeding [12,25].…”

“…Severe menorrhagia resulting in anemia or requiring transfusion was reported by 48-64% and 7-22% of women with type 1 vWD, respectively [18,24,25]. Women with vWD reported a significantly longer duration of bleeding and used a greater number of pads or tampons per cycle, compared to controls [18,25]. Ten to 26% of women underwent a hysterectomy to control excessive menstrual bleeding, prior to diagnosis in a substantial number of cases [4,18,24,25].…”

“…The risk of post-operative bleeding is not well defined, with the evidence limited to anecdotal reports and small case series. In three recent series summarizing the surgical outcomes of 29 hysterectomies, the rate of bleeding complications ranged from 0% to 60% [14,18,24]. The risk is likely higher in women with severe disease and in those who do not receive adequate prophylactic therapy.…”