Prevalence, burden and treatment effects of vaginal bleeding in women with (suspected) congenital platelet disorders throughout life: a cross‐sectional study

Abstract

Summary Congenital platelet disorders (CPDs) are rare bleeding disorders that are associated with mucocutaneous bleeds. However, data on vaginal bleeding in women with CPDs are scarce. A set of generic and bleeding‐specific questionnaires were used to evaluate the prevalence of vaginal bleeding, its impact on quality of life (QoL) and sexual functioning and the consequences for pregnancy, miscarriage and delivery in a cohort of women who were referred for diagnostic evaluation for CPDs. A total of 78 women included in the study were either diagnosed with a CPD (n = 35) or were clinically suspected of a CPD (n = 43). Heavy menstrual bleeding (HMB) was reported by a large proportion of women, which mainly started at menarche. In all, 76% of women received any kind of HMB treatment, often leading to surgical prodecures. HMB was shown to have a high impact on QoL, which improved upon treatment. Even though women reported that vaginal bleeding affects sexuality, this topic is not frequently discussed with physicians. Heavy blood loss frequently occurred after miscarriage/delivery, often requiring treatment. Women with (suspected) CPDs frequently encounter HMB, negatively impacting daily life and sexual functioning. Together with peripartum bleeding, these data highlight the burden of vaginal bleeding in CPDs and importance of adequate treatment.

The ISTH‐BAT score and outcomes after endometrial ablation in women with heavy menstrual bleeding

“…HMB is associated with a negative effect on QoL. 30,35 Several studies revealed social stress caused by all the hardships that accompany having a bleeding disorder before any treatment, and the radical choices they have to make as women. 30,35,36 ISTH-BAT ≥6 group, could have restricted our ability to identify statistically significant differences between the groups.…”

“…30,35 Several studies revealed social stress caused by all the hardships that accompany having a bleeding disorder before any treatment, and the radical choices they have to make as women. 30,35,36 ISTH-BAT ≥6 group, could have restricted our ability to identify statistically significant differences between the groups. In the total group more women reported amenorrhea at 2-5 years after initial EA (76%) compared to the women treated with EA for HMB described in the literature (50%).…”

Big picture initiatives in bleeding disorders

“…1 Heavy menstrual bleeding (HMB) is the most prevalent symptom in women with IBDs, whatever the underlying diagnosis : von Willebrand disease (VWD), inherited platelet disorders (IPDs), haemophilia carriers or other rare coagulation factor deficiencies. [2][3][4] These women can experience haemorrhagic ovarian cysts resulting sometimes in hematoperitoneum, 5 and are at higher risk of early and late postpartum haemorrhage (PPH), as illustrated by a 2-10 fold increased risk in women with VWD, the most frequent IBDs. 6 All these bleeding symptoms impair the quality of life by creating significant physical, emotional, sexual, social and financial burdens.…”

“…Although a late diagnosis is particularly deleterious for an adequate anticipation of menarche, too few clinicians consider IBDs in the differential diagnosis of adolescent and women with HMB. [15][16] Recent studies have also emphasized that HMB continues to pose significant ongoing management challenges following initial formal diagnosis of IBDs such as VWD 3 or IPDs. 44 Some gender specific issues may have been overlooked in women with IBD.…”

“…Women with IBDs are disproportionately affected, when compared to men, because of specific bleeding challenges related to menstruations and childbirth 1 . Heavy menstrual bleeding (HMB) is the most prevalent symptom in women with IBDs, whatever the underlying diagnosis : von Willebrand disease (VWD), inherited platelet disorders (IPDs), haemophilia carriers or other rare coagulation factor deficiencies 2–4 . These women can experience haemorrhagic ovarian cysts resulting sometimes in hematoperitoneum, 5 and are at higher risk of early and late postpartum haemorrhage (PPH), as illustrated by a 2–10 fold increased risk in women with VWD, the most frequent IBDs 6 .…”