Kadir R A, Economides D L, Sabin C A, Pollard D, Lee C A
University Department of Obstetrics and Gynaecology, The Royal Free Hospital, London, UK.
Haemophilia. 1999 Jan;5(1):40-8. doi: 10.1046/j.1365-2516.1999.00285.x.
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.
本研究评估了遗传性出血性疾病患者的月经失血情况及妇科问题。对116名女性进行了访谈，包括66名血管性血友病（vWD）患者、30名血友病携带者和20名因子XI（FXI）缺乏症患者，并获取了她们的妇科病史。同时查阅了她们的病例记录，并使用图片式失血评估图表（PBAC）客观评估月经失血情况。与年龄匹配的对照组（69名女性）进行了比较。vWD患者、血友病携带者和FXI缺乏症患者中，分别有74%、57%和59%被确诊为月经过多（PBAC评分>100），而对照组为29%（P = 0.001）。vWF:Ac≤30 IU dL-1的vWD患者PBAC评分高于水平较高者，但差异无统计学意义。然而，FXI缺乏症患者和血友病携带者的PBAC评分与疾病严重程度之间没有关联。与对照组相比，遗传性出血性疾病患者的月经持续时间显著更长（P = 0.001），大量出血情况显著更常见（P = 0.001）。然而，月经期间血块排出情况没有差异。47%的遗传性出血性疾病患者因月经过多咨询过家庭医生或妇科医生，36%接受过药物治疗，27%接受过手术治疗，包括10例子宫切除术。28例宫腔镜检查和/或刮宫术中，有4例术后出血。10例子宫切除术中，有5例报告了术后出血并发症。总之，月经过多是遗传性出血性疾病患者，尤其是vWD患者常见的主要问题。妇科医生和血液科医生提高对月经过多高患病率及可用治疗方案的认识，对于这些患者的最佳管理至关重要。在任何妇科手术中，无论手术多么微小，都需要进行适当的术前评估和止血控制，并与当地血友病中心合作，这对于将出血并发症风险降至最低至关重要。
