Division of Pediatric Hematology Oncology, University of Michigan Medical School, Ann Arbor, MI, United States.
Division of Pediatric Hematology Oncology, Stead Family Department of Pediatrics, University of Iowa Hospitals and Clinics, 1322 BT, 200 Hawkins Drive, Iowa City, IA 52242, United States.
J Pediatr Adolesc Gynecol. 2022 Dec;35(6):614-623. doi: 10.1016/j.jpag.2022.07.006. Epub 2022 Jul 10.
Reproductive tract bleeding is an underappreciated health care problem among adolescent and young adult (AYA) females with inherited bleeding disorders (IBDs) comprising von Willebrand disease, platelet disorders, hemophilia carriership, and rare factor deficiencies. IBDs are prevalent in women of all ages and have been detected in about 50% of women with menorrhagia or heavy menstrual bleeding (HMB) and about 20% of women with postpartum hemorrhage (PPH). The clinical spectrum of gynecologic and obstetric bleeding in AYA with IBDs ranges from HMB, ovulation bleeding, and surgical bleeding to miscarriages and life-threatening PPH. Reproductive tract bleeding adversely affects the quality of life of this patient population, in addition to causing substantial morbidity and mortality. Early diagnosis of IBDs offers the opportunity for timely intervention with hormones, hemostatic agents, and prophylaxis with factor concentrates, thereby improving outcomes. This review summarizes the epidemiology, pathophysiology, clinical manifestations, diagnostic approach, management, and prophylaxis for reproductive tract bleeding in AYA with IBDs. This review provides a multidisciplinary approach to the problem, which is critical to improve the outcomes of this patient population.
生殖道出血是青少年和年轻女性（AYA）中未被充分认识的健康问题，她们患有遗传性出血性疾病（IBD），包括血管性血友病、血小板疾病、血友病携带者和罕见的因子缺乏症。IBD 可发生于所有年龄段的女性，在月经过多或重度月经出血（HMB）的女性中约有 50%，在产后出血（PPH）的女性中约有 20%被检出。AYA 中 IBD 的妇科和产科出血的临床表现范围广泛，从 HMB、排卵出血和手术出血到流产和危及生命的 PPH。生殖道出血不仅会导致大量的发病率和死亡率，还会对这群患者的生活质量产生不利影响。早期诊断 IBD 提供了机会，以便及时用激素、止血剂和因子浓缩物进行预防，从而改善结局。本综述总结了 AYA 中 IBD 患者的生殖道出血的流行病学、病理生理学、临床表现、诊断方法、管理和预防。本综述提供了一种多学科方法来解决这个问题，这对改善这群患者的结局至关重要。
