Management of Inherited Bleeding Disorders in Pregnancy

doi:10.1111/1471-0528.14592

Cited by 78 publications

(7 citation statements)

References 244 publications

(357 reference statements)

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“…There is a paucity of studies describing management of pregnant women with FXI deficiency, and therefore guidelines are not evidence based. 10–16 Standard management of pregnant women with severe FXI deficiency is administration of FFP or FXI concentrate where available at the time of delivery, if women have a bleeding phenotype. Antifibrinolytics such as tranexamic acid (TXA) or epsilon aminocaproic acid (EACA) are also commonly used to decrease the risk of PPH in women with bleeding phenotype, regardless of partial or severe FXI deficiency.…”

Section: Introductionmentioning

confidence: 99%

“…Antifibrinolytics such as tranexamic acid (TXA) or epsilon aminocaproic acid (EACA) are also commonly used to decrease the risk of PPH in women with bleeding phenotype, regardless of partial or severe FXI deficiency. 15…”

Section: Introductionmentioning

confidence: 99%

“…Neuraxial anesthesia (epidural or spinal) in pregnant women with FXI deficiency is often avoided due to concern for spinal hematoma. 10,15 Several small retrospective studies have reported using neuraxial anesthesia during labor and delivery with no cases of spinal hematomas. 10,11,17–21 However, the administration of neuraxial anesthesia in pregnant women with FXI deficiency remains unsettled in clinical practice.…”

Section: Introductionmentioning

confidence: 99%

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Peri- and Postpartum Management of Patients With Factor XI Deficiency

Gerber

Klute

Chapin³

et al. 2019

Clin Appl Thromb Hemost

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Factor XI (FXI) deficiency is an uncommon autosomal disorder with variable bleeding phenotype, making peripartum management challenging. We describe our experience in pregnant women with FXI deficiency and identify strategies to minimize the use of hemostatic agents and increase utilization of neuraxial anesthesia. Electronic records of 28 pregnant women with FXI deficiency seen by a hematology service in an academic medical center from January 2006 to August 2018 were reviewed. Data on bleeding, obstetric history, peripartum management, and FXI activity were collected. Partial FXI deficiency was defined as >20 IU/dL and severe <20 IU/dL. Median FXI activity was 42 IU/dL (range <1-73 IU/dL), and median activated partial thromboplastin time was 32.2 seconds (range: 27.8-75 seconds). There were 64 pregnancies: 53 (83%) live births and 11 (17%) pregnancy losses. Postpartum hemorrhage occurred in 9 (17%) pregnancies. Antifibrinolytic agents and fresh frozen plasma were used only in women with severe deficiency (42% with bleeding and 17% with no bleeding phenotype, respectively). Neuraxial anesthesia was successfully administered in 32 (59%) deliveries. Most women with FXI deficiency have uncomplicated pregnancies and deliveries with minimal hemostatic support. Neuraxial anesthesia can be safely administered in most women.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Peri- and Postpartum Management of Patients With Factor XI Deficiency

Gerber

Klute

Chapin³

et al. 2019

Clin Appl Thromb Hemost

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“…One unit of FFP will increase all clotting factors by 2%-3%. 63 PCCs gives better recovery of factor X since it contains factors II, VII, IX, X and protein C. 64 PCC is preferable to FFP because it can be given quicker as an infusion and not associated with volume overload or transfusion-related acute lung injury.…”

Section: Discussionmentioning

confidence: 99%

Congenital Factor X deficiency in women: A systematic review of the literature

Spiliopoulos

Kadir

2019

Haemophilia

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Factor X deficiency (FXD) is a rare autosomal recessive bleeding disorder with a variable phenotypic severity. In women, heavy menstrual bleeding (HMB), recurrent ovulation bleeding with haemoperitoneum and bleeding complications in pregnancy such as retroplacental haematoma and postpartum haemorrhage have been reported. The aim of this review was to examine gynaecological problems and obstetric complications in women with congenital FXD. A total number of 49 relevant articles were identified, including 332 women, dating from 1960 to 2018. Heavy menstrual bleeding was reported in 72/284 (25%) women in total, 14/30 (47%) in case reports and 58/254 (23%) in 11 case series, 64% and 10% required blood products and blood transfusion, respectively. Haemoperitoneum from ovulation bleeding or ruptured haemorrhagic ovarian cyst requiring blood transfusion occurred in 8/322 (2.4%) women, six required surgical intervention, including oophorectomy in two. 31 pregnancies were reported in 19 women. There were four miscarriages (including a late miscarriage at 21 weeks). There was a high rate of preterm birth and neonatal death occurring in eight (30%) and three (11%) of pregnancies reaching viability stage. Postpartum haemorrhage (PPH) occurred in six (22%) of deliveries, one requiring hysterectomy. In conclusion, women with FXD are at an increased risk of heavy bleeding during menstruation and ovulation as well as adverse pregnancy outcome and postpartum haemorrhage. Collaboration in a multidisciplinary team including an obstetrician/gynaecologist, a perinatologist and a haematologist is necessary for the prevention and management of these complications.

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“…The Royal College of Anaesthetists in the UK makes allotting time for antenatal assessment one condition for accreditation of anaesthetic units [16]. There is similar guidance from Royal College of Obstetrics and Gynaecology [9, 17]. Making anaesthetic antenatal assessment facilities a requirement or at least strongly recommended by national authorities will likely to increase uptake.…”

Section: Main Textmentioning

confidence: 99%

Antenatal anaesthesia clinics – the way forward?

Plaat¹,

Harris²

2018

Isr J Health Policy Res

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Specialist antenatal clinics are increasingly being used to enable anaesthetists to evaluate pregnant women with co-morbidities and those at high risk of obstetric complications. In this journal a team from Israel describe the process of setting up and running such a clinic over a 14 year period. One of the challenges they identify was the limited referral of high risk women. Based on UK and US literature, the use of structured referral tools, clear criteria for referral and regular antenatal multidisciplinary meetings may help to address this.

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Management of Inherited Bleeding Disorders in Pregnancy

Cited by 78 publications

References 244 publications

Peri- and Postpartum Management of Patients With Factor XI Deficiency

Peri- and Postpartum Management of Patients With Factor XI Deficiency

Congenital Factor X deficiency in women: A systematic review of the literature

Antenatal anaesthesia clinics – the way forward?

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