Factor X deficiency and pregnancy: case report and counselling

Teixeira, Pedro Soares; Oliveira, Paulo H.; Guerra, João Carlos Campos; Hamerschlak, Nelson; Colombini, Marjorie Paris; Kalil, Renato A. K.

doi:10.1111/hae.12506

Cited by 2 publications

(8 citation statements)

References 14 publications

(14 reference statements)

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“…In total, 332 women were identified; 30 women in 30 case reports (Table ) and 302 women in 19 case series. In the case reports, the FX coagulant activity was <0.01 IU/mL in 14 women, 0.01‐0.05 IU/mL in 8 women and >0.05 IU/mL in four women, and in four women, the severity of FX deficiency was not mentioned . In the case series, 63 patients had FX coagulant activity ≤0.01 IU/mL, 95 patients >0.01 IU/mL (range 0.01‐0.26) and in the rest FX level was not reported.…”

Section: Resultsmentioning

confidence: 93%

“…Heavy menstrual bleeding was a common bleeding symptom reported in 72/284 (25%) in total, 14/30 (47%) in case reports, and 58/254 (23%) in 11 case series (Table ). Semi‐objective assessment of HMB in terms of pictorial blood assessment chart was not reported.…”

Section: Resultsmentioning

confidence: 97%

“…In this review, 31 pregnancies were reported in 19 women . 4/31 (13%) resulted in miscarriage and 27/31(87%) pregnancies reached viability stage (>24 weeks) . Of the latter, 8/27 (30%) pregnancies were delivered preterm (<37 weeks) and 16/27 (59%) at term (≥37 weeks) .…”

Section: Pregnancymentioning

confidence: 88%

“…In this review, 31 pregnancies were reported in 19 women . 4/31 (13%) resulted in miscarriage and 27/31(87%) pregnancies reached viability stage (>24 weeks) .…”

Section: Pregnancymentioning

confidence: 89%

“…4/31 (13%) resulted in miscarriage and 27/31(87%) pregnancies reached viability stage (>24 weeks) . Of the latter, 8/27 (30%) pregnancies were delivered preterm (<37 weeks) and 16/27 (59%) at term (≥37 weeks) . The gestational age at delivery was not reported in three cases .…”

Section: Pregnancymentioning

confidence: 98%

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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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Section: Resultsmentioning

confidence: 93%

Section: Resultsmentioning

confidence: 97%

Section: Pregnancymentioning

confidence: 88%

“…In this review, 31 pregnancies were reported in 19 women . 4/31 (13%) resulted in miscarriage and 27/31(87%) pregnancies reached viability stage (>24 weeks) .…”

Section: Pregnancymentioning

confidence: 89%

Section: Pregnancymentioning

confidence: 98%

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

Spiliopoulos

Kadir

2019

Haemophilia

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Management of rare inherited bleeding disorders: Proposals of the French Reference Centre on Haemophilia and Rare Coagulation Disorders

Trossaërt¹,

Chamouard

Biron‐Andréani

et al. 2023

European J of Haematology

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Introduction: The rare coagulation disorders may present significant difficulties in diagnosis and management. In addition, considerable inter-individual variation in bleeding phenotype is observed amongst affected individuals, making the bleeding risk difficult to assess in affected individuals. The last international recommendations on rare inherited bleeding disorders (RIBDs) were published by the United Kingdom Haemophilia Centre Doctors' Organisation in 2014. Since then, new drugs have been marketed, news studies on surgery management in patients with RIBD have been published, and new orphan diseases have been described. Aim: Therefore, the two main objectives of this review, based on the recent recommendations published by the French Reference Centre on Haemophilia and Rare Bleeding Disorders, are: (i) to briefly describe RIBD (clinical presentation and diagnostic work-up) to help physicians in patient screening for the early detection of such disorders; and (ii) to focus on the current management of acute haemorrhages and long term prophylaxis, surgical interventions, and pregnancy/delivery in patients with RIBD.

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Factor X deficiency and pregnancy: case report and counselling

Cited by 2 publications

References 14 publications

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

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

Management of rare inherited bleeding disorders: Proposals of the French Reference Centre on Haemophilia and Rare Coagulation Disorders

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