title: Congenital factor V and VIII deficiency in women: a systematic review of literature and report of two new cases.
abstract: Factor V and factor VIII deficiency (F5F8D) is a rare congenital bleeding disorder. There is a paucity of data in the literature about obstetric and gynaecological problems in women affected by F5F8D. The aim of this review was to examine obstetric complications and gynaecological problems in women with congenital F5F8D and present two new cases. An electronic search was performed to identify the published literature on PUBMED, MEDLINE and EMBASE databases using the following keywords 'congenital factor V and factor VIII deficiency' and 'women or pregnancy'. A total of 23 relevant articles were found and included in this systematic review: 15 case reports and 10 case series dating from 1976 to 2015. A total number of 86 women were identified. Heavy menstrual bleeding was the most common bleeding symptom in women (49%). Recurrent ovulation bleeding and haemorrhagic ovarian cyst were reported in three women. Nineteen pregnancies were reported (including our two case reports). There were no miscarriages. Postpartum bleeding occurred in six (32%) deliveries. In conclusion, data are very limited on gynaecological and obstetric problems in women with F5F8D. Heavy menstrual bleeding is a common problem. There is also an increased risk of postpartum haemorrhage. Close collaboration between haemophilia, obstetric and gynaecological teams is important to prevent and manage obstetric and gynaecological bleeding complications.
authors.affiliation: Katharine Dormandy Haemophilia Centre and Department of Obstetrics & Gynaecology, Royal Free Hospital, London, UK.
authors.forename: Dimitrios
authors.initials: D
authors.lastname: Spiliopoulos
authors.forename: Rezan A
authors.initials: RA
authors.lastname: Kadir
mesh_terms: D000293:Adolescent; D000328:Adult; D005166:Factor V Deficiency / Q000097:blood / Q000150:complications*; D005260:Female; D006467:Hemophilia A / Q000097:blood / Q000150:complications*; D006470:Hemorrhage / Q000097:blood / Q000209:etiology*; D006801:Humans; D008597:Menstrual Cycle; D006473:Postpartum Hemorrhage / Q000097:blood / Q000209:etiology; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000097:blood / Q000209:etiology*