title: Gynaecological and obstetrical bleeding in women with factor XI deficiency - a systematic review.
abstract: BACKGROUND
  Menstrual bleeding, pregnancy and delivery present an intrinsic haemostatic challenge to women with bleeding disorders such as factor XI (FXI) deficiency.
  
  
  OBJECTIVE
  To provide a systematic overview of studies on gynaecological and obstetrical bleeding problems in women with FXI deficiency.
  
  
  METHODS
  We searched MEDLINE, EMBASE and the Cochrane library for studies that present original data on the incidence of and treatment options for gynaecological and obstetrical bleeding in FXI-deficient women.
  
  
  RESULTS
  We identified 27 studies, including a total of 372 women with FXI deficiency. All studies were observational, no interventional treatment studies were found. Most patients had a mild deficiency (FXI ≥ 20 IU dL-1). Heavy menstrual bleeding (HMB) was reported in 7-67%. In 7/19 (37%) women who underwent gynaecological procedures, a bleeding complication occurred, including in 2/7 hysterectomies (29%). About 3-20% of reported pregnancies ended in a miscarriage; of these miscarriages 0-25% (4/23 miscarriages) were complicated by bleeding. Terminations of pregnancies (TOP) were complicated by bleeding in 4 out of 11 cases (36%). In 90 out of 498 (18%) deliveries a postpartum haemorrhage (PPH) was reported, ranging from 0 to 50% in individual studies. In 21% (66/321) of deliveries, prophylaxis was given. This was associated with 9% (6/66) PPH, compared to 19% in deliveries without prophylaxis (84/432). Epidural analgesia was performed without complications in 44 patients.
  
  
  CONCLUSIONS
  Women with FXI deficiency have a clearly increased risk of HMB, and of bleeding complications after miscarriage, TOP and delivery. No high quality data are available regarding prophylactic treatment.
authors.affiliation: Division of Haemostasis and Thrombosis, Department of Haematology, University of Groningen University Medical Centre Groningen, Groningen, The Netherlands.|Department of Obstetrics and Gynaecology, University of Groningen University Medical Centre Groningen, Groningen, The Netherlands.
authors.forename: S
authors.initials: S
authors.lastname: Wiewel-Verschueren
authors.affiliation: Department of Obstetrics and Gynaecology, University of Groningen University Medical Centre Groningen, Groningen, The Netherlands.
authors.forename: I J
authors.initials: IJ
authors.lastname: Arendz
authors.affiliation: Department of Obstetrics and Gynaecology, University of Groningen University Medical Centre Groningen, Groningen, The Netherlands.|Isala Clinics Department of Obstetrics and Gynaecology, Zwolle, The Netherlands.
authors.forename: H
authors.initials: H
authors.lastname: M Knol
authors.affiliation: Division of Haemostasis and Thrombosis, Department of Haematology, University of Groningen University Medical Centre Groningen, Groningen, The Netherlands.
authors.forename: K
authors.initials: K
authors.lastname: Meijer
keywords: FXI deficiency; gynaecological bleeding; heavy menstrual bleeding; obstetrical bleeding