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少女月经过多和出血性疾病。

Menorrhagia and bleeding disorders in adolescent females.

机构信息

Medical Thrombosis and Haemophilia treatment Center, Duisburg, Germany.

出版信息

Hamostaseologie. 2012;32(1):45-50. doi: 10.5482/ha-1181. Epub 2011 Nov 29.

Abstract

In women, von Willebrand disease (VWD) is the most common inherited bleeding disorder. Since VWD and other inherited bleeding disorders are autosomal disorders, they affect women and men. Menorrhagia, or heavy menstrual bleeding (HMB), is the most common symptom of women with bleeding disorder experience. Objectively, it is defined as bleeding that lasts for more than seven days or results in the loss of more than 80 ml of blood per menstrual cycle. The prevalence of menorrhagia in a woman with a bleeding disorder ranges from 32 to 100% in patients with VWD, from 5 to 98% in patients with a platelet dysfunction and from 35 to 70% in women with a rare factor deficiency. A detailed history and a careful physical exam are the first steps towards a diagnosis in adolescents, adding a PBAC>100 increased the sensitivity of the screening tool further to 95%. Laboratory testing should be made at the time of menstrual bleeding in an effort to capture the lowest level of VWF:Ag and FVIII:C. Treatment options for menorrhagia in VWD: (1) antifibrinolytic therapy with tranexamic acid, (2) the non-transfusional agent desmopressin (DDAVP), (3) purified blood products that contain factor VIII and VWF concentrated from plasma and (4) hormonal preparations.

摘要

在女性中,血管性血友病(VWD)是最常见的遗传性出血性疾病。由于 VWD 和其他遗传性出血性疾病是常染色体疾病,因此它们会影响女性和男性。月经过多,即月经大出血(HMB),是患有出血性疾病的女性最常见的症状。客观上,它被定义为持续超过 7 天或导致每个月经周期失血超过 80 毫升的出血。在 VWD 患者中,月经过多的患病率为 32%至 100%,在血小板功能障碍患者中为 5%至 98%,在罕见因子缺乏症的女性中为 35%至 70%。详细的病史和仔细的体格检查是青少年诊断的第一步,将 PBAC>100 添加到筛选工具中可进一步将敏感性提高到 95%。应在月经出血时进行实验室检测,以尽量捕捉到 VWF:Ag 和 FVIII:C 的最低水平。VWD 月经过多的治疗选择:(1)使用氨甲环酸的抗纤维蛋白溶解疗法,(2)非输血剂去氨加压素(DDAVP),(3)从血浆浓缩的包含因子 VIII 和 VWF 的纯化血液制品,以及(4)激素制剂。

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