Silva Carla Donato, Geraldes Fernanda, Silva Isabel Santos
Department of Obstetrics and Gynecology, Bissaya Barreto Maternity Hospital, Coimbra, Portugal.
BMJ Case Rep. 2013 Apr 30;2013:bcr2013008833. doi: 10.1136/bcr-2013-008833.
The authors describe a case of an adolescent with type III von Willebrand disease and severe menorrhagia since menarche. Antifibrinolytic, hormonal (estroprogestative pill in high doses, etonogestrel implant and gonadotropin-releasing hormone agonist goserelin) and Von Willebrand Factor/Factor VIII replacement therapies were prescribed to the patient, but symptomatic control was only obtained with high doses of VWF/FVIII twice a week. In March 2012, a levonorgestrel intrauterine system was inserted in a 14-year-old. At present, the patient is asymptomatic without regular prophylaxis (VWF/FVIII replacement therapy) and has had a remarkable improvement in her quality of life.
作者描述了一例自初潮起就患有III型血管性血友病且月经过多的青少年病例。给该患者开了抗纤溶、激素(高剂量雌孕激素联合避孕药、依托孕烯植入剂和促性腺激素释放激素激动剂戈舍瑞林)以及血管性血友病因子/凝血因子VIII替代疗法,但仅通过每周两次高剂量的血管性血友病因子/凝血因子VIII才获得了症状控制。2012年3月,一名14岁的患者植入了左炔诺孕酮宫内节育系统。目前,该患者在未进行常规预防(血管性血友病因子/凝血因子VIII替代疗法)的情况下无症状,生活质量有了显著改善。