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Reproductive Tract Bleeding in Adolescent and Young Adult Females with Inherited Bleeding Disorders: An Underappreciated Problem

      Abstract

      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.

      Abbreviations:

      Ang-1 (Angiopoietin-1), APH (Ante-partum hemorrhage), ATHN (American Thrombosis and Hemostasis Network), AYA (Adolescents and young adults), BMI (Body mass index), CDC (Centers for Disease Control), CHC (Combined hormonal contraception), COCs (Combined oral contraceptives), DDAVP (l-desamino-8-D-arginine-vasopressin), DIC (Disseminated intravascular coagulation), DMPA (Depot medroxy-progesterone acetate), FFP (Fresh frozen plasma), FUDC (Female Universal Data Collection), HESC (Human endometrial stromal cells), HMB (Heavy menstrual bleeding), HTC (Hemophilia treatment center), IBDs (Inherited bleeding disorders), IUD (Intrauterine device), ISTH-BAT (International Society of Haemostasis and Thrombosis Bleeding Assessment Tool), MMP (Matrix metalloprotease), PAI-1 (Plasminogen activator inhibitor-1), PAR-2 (Protease activating receptor-2), PBAC (Pictorial bleeding assessment chart), PCC (Prothrombin complex concentrate), PCOS (Polycystic ovarian syndrome), PFA (Platelet function analyzer), PFD (Platelet function defect), PPH (Postpartum hemorrhage), PT (Prothrombin time), PTT (Partial thromboplastin time), RFD (Rare factor deficiencies), TEG (Thrombo-elastography), TF (Tissue factor), TGA (Thrombin generation assay), t-PA (Tissue plasminogen activator), TXA (Tranexamic acid), UDC (Universal Data Collection), VEGF (Vascular endothelial growth factor), VTE (Venous thromboembolism), VWD (Von Willebrand disease), VWF (Von Willebrand factor)
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