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.
MYH9-related disease (MYH9-RD) is a rare autosomal dominant thrombocytopenia characterized by giant platelets, thrombocytopenia and granulocyte Döhle body-like cytoplasmic inclusion bodies and can be accompanied by deafness, cataracts and nephritis, based on specific genotype. 1 Mild to severe bleeding diathesis is one of its common clinical manifestations. 1 Bleeding problems encountered by women, including heavy menstrual bleeding (HMB) and postpartum haemorrhage (PPH), can be challenging to manage. 2,3 The existing literature on women-specific bleeding in MYH9-RD is limited; an overview of data on HMB in these women is not yet available. Data on pregnancy outcome are restricted to one review published in 2013, confined to May-Hegglin anomaly and only summarizing case reports/series, while a large cohort study has been published in 2014. 4,5 This systematic review therefore aims to summarize all published data on menstrual and obstetrical bleeding and the respective management strategies for women with MYH9-RD. The protocol was registered on PROSPERO (registration number CRD42018115116) and adhered to Cochrane methodology. 6,7 Electronic databases were searched to identify articles with original patient data on HMB or pregnancy in women with MYH9-RD (Appendix S1). Two reviewers independently screened the titles, abstracts, full texts and reference lists to identify potentially relevant articles. One reviewer conducted data extraction, using a standard
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