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Women with bleeding disorders: Clinical and psychological issues

Inherited bleeding disorders show wide variation in severity and prevalence but are described in all populations. Some are related to defects in primary haemostasis such as platelet function disorders or von Willebrand disease and others such as haemophilia A and B, and factor XI deficiency are due to defects in secondary haemostasis. Inherited bleeding disorders affect both men and women but this review article aims to discuss the impact of bleeding disorders in women. While it has been recognized that women are affected by bleeding disorders such as von Willebrand disease (VWD) and other rare bleeding disorders, arguably until recently, the dominant clinical and research focus in the field of bleeding disorders has focused predominantly on management of males with haemophilia A and B. Exciting advances in care for people with haemophilia should not overshadow the increasing attention that has been given to the impact of bleeding disorders on women. It is well recognized that autosomal dominant or recessive bleeding disorders such VWD and FXI, FXIII deficiency affect both males and females but that females with the gene for haemophilia A or B also manifest bleeding symptoms is under recognized. A significant number of these females have clotting factor levels that would classify them as having mild, moderate and even severe haemophilia. This review will discuss the impact of bleeding disorders on women summarizing important genetic aspects of disorders that impact on the severity of women’s symptoms, outline approach to management of bleeding issues specific to women such as heavy menstrual bleeding and care in pregnancy and childbirth, and also discuss some of the psychological impacts for women with bleeding disorders. 2 | UNDERSTANDING THE GENETIC S OF BLEEDING DISORDERS