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Reproductive Tract Bleeding in Adolescent and Young Adult Females with Inherited Bleeding Disorders: An Underappreciated Problem
Journal of Pediatric and Adolescent Gynecology ( IF 1.8 ) Pub Date : 2022-07-10 , DOI: 10.1016/j.jpag.2022.07.006
Neeraja Swaminathan 1 , Anjali Sharathkumar 2 , Tazim Dowlut-McElroy 3
Affiliation  

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.



中文翻译:

患有遗传性出血性疾病的青春期和年轻成年女性的生殖道出血:一个被低估的问题

生殖道出血是患有遗传性出血性疾病 (IBD) 的青春期和年轻成年 (AYA) 女性的一个未被充分认识的医疗保健问题,这些疾病包括血管性血友病、血小板疾病、血友病携带和罕见因子缺乏症。IBD 在所有年龄段的女性中都很普遍,并且已在约 50% 的月经过多或经血过多 (HMB) 的女性和约 20% 的产后出血 (PPH) 的女性中检测到。AYA 伴 IBD 的妇科和产科出血的临床范围从 HMB、排卵期出血、手术出血到流产和危及生命的 PPH。生殖道出血除了导致大量的发病率和死亡率外,还不利地影响该患者群体的生活质量。IBD 的早期诊断为及时干预激素、止血剂和使用凝血因子预防提供了机会,从而改善结果。本综述总结了伴有 IBD 的 AYA 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。这篇综述提供了解决该问题的多学科方法,这对于改善该患者群体的结果至关重要。

更新日期:2022-07-10
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