Severe factor X deficiency in pregnancy: case report and review of the literature

Romagnolo, C; Burati, S; Ciaffoni, S; Fattori, E; Franchi, Massimo Piergiuseppe; Zanon, Ezio; Girolami, Antonio

doi:10.1111/j.1365-2516.2004.01012.x

Cited by 29 publications

(42 citation statements)

References 12 publications

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“…The review of the literature has discovered only a few pregnancies in patients with FII and FX deficiencies [5, 6, 8,23,24,25,26]. The rarity of these two disorders may fully justify the finding.…”

Section: Discussionmentioning

confidence: 96%

Pregnancy and Oral Contraceptives in Congenital Bleeding Disorders of the Vitamin K- Dependent Coagulation Factors

Girolami

Randi²,

Ruzzon³

et al. 2006

Acta Haematol

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Pregnancies and deliveries represent important hemostatic challenges for congenital coagulation disorders. The same is true for the assumption of oral contraceptives. Available information mainly deals with von Willebrand’s disease, factor XI (FXI) deficiency and carriers of hemophilia A. Data concerning patients with congenital prothrombin complex factor deficiencies are very scanty. In the present study, data of a total of 27 women are presented, 11 patients with homozygous or double heterozygous deficiencies of FII, FVII and FX, together with 16 cases of hemophilia B carriers. The patients with FII, FVII or FX defects had a total of 14 pregnancies and often needed transfusion therapy. Proper management resulted in a decrease in postpartum bleeding and satisfactory fetal outcome. Elective cesarean delivery seems indicated only in recent years. Carriers of hemophilia B had a total of 19 pregnancies but showed no bleeding and needed no substitutive therapy. Searching the literature, we discovered only 9 additional patients with prothrombin deficiency or FX deficiency, having a total of 16 pregnancies. On the contrary, there were at least 17 additional patients with FVII deficiency, with a total of 21 pregnancies. The management of the diseases has been variable, but in substantial agreement with the personal observations. Oral contraceptive therapy was administered in some of our patients and in a few additional cases described in the literature. Medication was always well tolerated and patients who took it for a long period of time showed a decrease in menometrorrhagia and an improvement in hematocrit levels. This led to a decrease in transfusional needs and to improved general conditions.

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Section: Discussionmentioning

confidence: 96%

Pregnancy and Oral Contraceptives in Congenital Bleeding Disorders of the Vitamin K- Dependent Coagulation Factors

Girolami

Randi²,

Ruzzon³

et al. 2006

Acta Haematol

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“…Prophylaxis was given during delivery, and postpartum period in 14 pregnancies and 16 pregnancies did not receive prophylaxis during this period (FX levels were < 0.01 IU/mL in one, 0.06 IU/mL in another one, 0.4 IU/mL in a third case and in another levels were not reported), and in one case, there was no record of prophylaxis administered …”

Section: Postnatal Complicationsmentioning

confidence: 99%

“…In eight pregnancies, PCC was used as prophylaxis (with doses varying from 1500 to 2000 U given twice or three times/week reported in three cases), FFP was given for prophylaxis in three and pdFX used in two cases; in one case, the product used was not specified…”

Section: Postnatal Complicationsmentioning

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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“…Factor X levels increase during pregnancy (in women with normal FX levels at the start of pregnancy) [13] but women with severe FX deficiency and a history of adverse outcome in pregnancy may benefit from aggressive replacement therapy [37,38].…”

Section: Management Of Severe Factor X Deficiency In Pregnancymentioning

confidence: 99%

Factor X and Factor X Deficiency

Perry

2014

Textbook of Hemophilia

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Severe factor X deficiency in pregnancy: case report and review of the literature

Cited by 29 publications

References 12 publications

Pregnancy and Oral Contraceptives in Congenital Bleeding Disorders of the Vitamin K- Dependent Coagulation Factors

Pregnancy and Oral Contraceptives in Congenital Bleeding Disorders of the Vitamin K- Dependent Coagulation Factors

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

Factor X and Factor X Deficiency

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