Vijapurkar M, Mota L, Shetty S, Ghosh K
National Institute of Immunohaematology (ICMR), KEM Hospital, Parel, Mumbai, India.
Haemophilia. 2009 Jan;15(1):199-202. doi: 10.1111/j.1365-2516.2008.01894.x. Epub 2008 Sep 18.
At this centre, 130 women with rare bleeding disorders (RBD) were investigated over the past 15 years. Fifty patients were above the age of menarche (age of menarche in India is 10 years). Of these 44 presented with menorrhagia. Other complications in these patients involved bleeding because of ruptured graffian follicle (1), severe haemorrhage following caesarean section (1), recurrent pregnancy losses (3), hysterectomy to control menorrhagia (2), laser ablation of endometrium (1) and irradiation of ovary (1). Three patients voluntarily chose to remain unmarried because of the problems associated with menorrhagia which they assumed will interfere with married life. All the 45 patients had iron deficiency anaemia. The spectrum of RBD in these patients comprised Glanzmann's thrombasthenia (17), Bernard-Soulier syndrome (2), storage pool disorder (2), factor V (FV) deficiency (3), combined FV and factor VIII deficiency (5), factor XI deficiency (3), factor XIII deficiency (1), factor X deficiency (5), factor VII deficiency (2), alpha(2)-antiplasmin deficiency (1) and afibrinogenemia (3). RBD in women is diagnosed late and often they are not optimally managed hence suffer both iatrogenic and non-iatrogenic complications in this country.
在该中心,过去15年里对130名患有罕见出血性疾病(RBD)的女性进行了调查。50名患者初潮年龄以上(印度初潮年龄为10岁)。其中44名表现为月经过多。这些患者的其他并发症包括卵泡破裂出血(1例)、剖宫产术后严重出血(1例)、复发性流产(3例)、因月经过多行子宫切除术(2例)、子宫内膜激光消融术(1例)和卵巢照射(1例)。3名患者因月经过多相关问题而自愿选择不结婚,她们认为这会干扰婚后生活。所有45名患者均患有缺铁性贫血。这些患者的RBD谱包括Glanzmann血小板无力症(17例)、Bernard-Soulier综合征(2例)、贮存池病(2例)、因子V(FV)缺乏症(3例)、FV和因子VIII联合缺乏症(5例)、因子XI缺乏症(3例)、因子XIII缺乏症(1例)、因子X缺乏症(5例)、因子VII缺乏症(2例)、α(2)-抗纤溶酶缺乏症(1例)和无纤维蛋白原血症(3例)。在该国,女性RBD诊断较晚,且往往未得到最佳治疗,因此会遭受医源性和非医源性并发症。