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Keywords:

  • menstrual blood loss;
  • menorrhagia;
  • von Willebrand's disease;
  • carriers of haemophilia;
  • factor XI deficiency

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 (= 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 (= 0.001) and episodes of flooding was significantly more common (= 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.