Gynecology and Obstetrics Department, Faculty of Medicine Kasr Al Aini University Hospitals- El Manyal, Cairo, Egypt.
Haemophilia. 2014 Mar;20(2):238-43. doi: 10.1111/hae.12335. Epub 2013 Nov 20.
von Willebrand disease (VWD) is frequently ignored as a cause of menorrhagia. We investigated Egyptian women complaining of heavy menstrual bleeding (HMB) and/or other bleeding symptoms to detect potential VWD cases. Seventy-five female patients complaining of HMB and/or bleeding symptoms and 38 age-matched healthy female controls went through a family history questionnaire, a physical examination and were evaluated for bleeding score, pictorial blood assessment chart (PBAC), complete blood count, serum ferritin, blood group, prothrombin time, activated partial thromboplastin time, factor VIII (FVIII) activity, von Willebrand factor (VWF) ristocetin cofactor (RCo) activity, antigen (Ag), and RCo/Ag ratio. Sixty-eight of 75 patients presented with HMB, out of which 46 had no organic pathology and 7 presented other bleeding symptoms. Six patients were diagnosed with VWD, three with HMB, two with other bleeding symptoms and one with family history of VWD. Two related VWD patients were diagnosed in the control group. There were significant differences in bleeding and PBAC scores, ferritin level, FVIII activity, VWF:RCo and VWF:Ag between VWD patients and controls. This study indicated a high prevalence of VWD among patients with HMB without organic pathology (6.5%) and demonstrated the sensitivity of diagnostic parameters of VWD patients in an outreach campaign. The inexpensive bleeding and PBAC scoring systems are valuable to exclude cases without objective bleeding symptoms. Raising gynaecologists awareness about hereditary bleeding disorders is important to ensure a proper diagnosis and possible referral of these patients. Management of these patients with comprehensive medical care services under a multidisciplinary team would be ideal.
血管性血友病(VWD)常被忽视为月经过多的原因。我们调查了抱怨月经过多(HMB)和/或其他出血症状的埃及女性,以发现潜在的 VWD 病例。75 名抱怨 HMB 和/或出血症状的女性患者和 38 名年龄匹配的健康女性对照者接受了家族史问卷、体格检查,并进行了出血评分、图像血液评估图表(PBAC)、全血细胞计数、血清铁蛋白、血型、凝血酶原时间、活化部分凝血活酶时间、VIII 因子(FVIII)活性、血管性血友病因子(VWF)瑞斯托霉素辅因子(RCo)活性、抗原(Ag)和 RCo/Ag 比值的评估。75 名患者中有 68 名出现 HMB,其中 46 名无器质性病变,7 名出现其他出血症状。6 名患者被诊断为 VWD,3 名 HMB,2 名其他出血症状,1 名有 VWD 家族史。对照组中有 2 名相关的 VWD 患者。VWD 患者和对照组在出血和 PBAC 评分、铁蛋白水平、FVIII 活性、VWF:RCo 和 VWF:Ag 方面存在显著差异。本研究表明,无器质性病变的 HMB 患者中 VWD 的患病率较高(6.5%),并在普及活动中证明了 VWD 患者诊断参数的敏感性。廉价的出血和 PBAC 评分系统对于排除没有客观出血症状的病例很有价值。提高妇科医生对遗传性出血性疾病的认识对于确保这些患者的正确诊断和可能的转介非常重要。在多学科团队下提供全面的医疗服务对这些患者进行管理将是理想的。