In the childbearing years, hormonal therapy or hormonal changes in the menstrual cycle or the puerperium may be complicated by bleeding or thrombosis; however, among women with congenital disorders of hemostasis and thrombosis, the risk of bleeding or thrombosis may be increased. In women with congenital bleeding disorders, heavy menstrual bleeding is the most common bleeding symptom, and postpartum hemorrhage occurs despite treatment. Given the limitations of current therapy and the associated medical and psychological burden in women with bleeding disorders, better treatment approaches are needed to improve health outcomes and quality of life. In women with congenital thrombotic disorders, thromboembolism may complicate exogenous hormonal therapy and endogenous hormonal change during pregnancy and procedures, but risk differs by type of thrombophilia, procedure, time at risk, and thrombosis risk factors, all of which affect management. In this article, I shall consider a case-based discussion of current issues in women with congenital bleeding and clotting disorders, including heavy menstrual bleeding in a woman with VWD, postpartum hemorrhage risk in VWD, and thrombosis risk with oocyte retrieval in a woman with factor V Leiden and past thromboembolism. The goals are to review bleeding or thrombosis risk in these cases, current data, limitations of current treatment guidelines, and areas for future study.

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