Management of pregnancy in women with factor VII deficiency: A case series

Lee, Eun‐Ju; Burey, L.; Abramovitz, Sharon; DeSancho, Maria T.

doi:10.1111/hae.14086

Cited by 21 publications

(58 citation statements)

References 18 publications

(30 reference statements)

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“…Iron‐deficiency anemia was observed in 17 women (15%), associated with Plummer–Vinson syndrome in one case; 10,15,16,20,25,26,33,35,44 15 of these women had HMB. Only two women with anemia did not report HMB, including one who only exhibited anemia during pregnancy 12,16 …”

Section: Resultsmentioning

confidence: 98%

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Reproductive health and hemostatic issues in women and girls with congenital factor VII deficiency: A systematic review

Kadir

Gomez

2022

Journal of Thrombosis and Haemostasis

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Background Congenital factor VII (FVII) deficiency is an inherited bleeding disorder, with heterogenous bleeding symptoms. Women with FVII deficiency face hemostatic challenges during menstruation, ovulation, and childbirth. This systematic review evaluated prevalence and management of bleeding symptoms associated with gynecological and obstetric issues in women with FVII deficiency. Methods Databases (BIOSIS Previews, Current Contents Search, Embase, and MEDLINE) were searched for studies reporting FVII deficiency and gynecological or obstetric issues in women. Articles were screened using Joanna Briggs Institute checklists and relevant data extracted. Results One hundred fourteen women were identified from 62 publications. Forty‐six women had severe deficiency (FVII:C < 5% or <5 IU/dl). Heavy menstrual bleeding (HMB) was the most common bleeding symptom (n = 94; 82%); hospitalization and urgent medical/surgical interventions for acute HMB episodes were required in 16 women (14%). Seven women reported ovarian bleeding (6%); other bleeding symptoms varied. Patient management was inconsistent and included hemostatic and hormonal treatments. Only four women (7%) reporting vaginal bleeding during pregnancy. Postpartum hemorrhage (PPH) occurred following 12/45 deliveries (27%; 5 [42%] requiring blood transfusion) and was not necessarily prevented by prophylaxis (8 women). Conclusion Women with congenital FVII deficiency have an increased risk of HMB, ovarian bleeding, and PPH, impacting quality of life. Recognition of a bleeding disorder as the cause is often delayed. Management of bleeding complications is heterogeneous due to lack of treatment guidelines. Harmonizing severity classification of FVII deficiency may help standardize treatment strategies and development of specific guidelines for these women.

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Section: Resultsmentioning

confidence: 98%

“…plasma-derived FVII concentrate (one case), 18 or inducing uterine contraction (four cases), 16,17,57 with uterotonic agents including oxytocin infusion, and intramuscular ergotamine and prostaglandins.…”

Section: Bleeding During Deliverymentioning

confidence: 99%

Reproductive health and hemostatic issues in women and girls with congenital factor VII deficiency: A systematic review

Kadir

Gomez

2022

Journal of Thrombosis and Haemostasis

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“…Therefore, the authors recommend that rFVIIa should be made available in the case of hemorrhage or surgical intervention, but not as mandatory prophylaxis. In the research based on a series of five patients, Lee et al suggested that the management of delivery for women with FVII deficiency should be addressed on a case-by-case basis and at centers with expertise in rare bleeding disorders [ 30 ]. In two other recently published articles, peripartum prophylaxis was strongly recommended [ 31 , 32 ].…”

Section: Discussionmentioning

confidence: 99%

Thromboelastometry as an Ancillary Tool for Evaluation of Coagulation Status after rFVIIa Therapy in a Pregnant Woman with Severe Hypoproconvertinemia—A Case Series and Review of the Literature

Krzyżanowski

Gęca

Sokołowska

et al. 2022

IJERPH

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Introduction: Factor VII (FVII) deficiency is a rare hemorrhagic diathesis. In females, heavy menstrual and postpartum bleeding can appear as a consequence of its deficiency. Supplementation of the recombinant FVIIa is widely accepted. The supplementation effect in FVII-deficient subjects is difficult to predict, and severe hemorrhage has been described even when FVII levels after supplementation were within normal ranges. The aim of this report is to present the application of thromboelastometry to control the coagulation status in a patient with severe FVII deficiency during pregnancy and delivery, supplemented by rFVIIa per protocol complicated with life-threatening venous thromboembolism. Methods: Rotational thromboelastometry (ROTEM) was performed in 16 pregnant women: in one 28 year old primigravida at 35 weeks of pregnancy with congenital FVII deficiency after rFVIIa administration and 15 healthy women at 38 gestational weeks. The results were compared. Results: The thromboelastometry results showed significant shortening of the clotting time in the extrinsic and the intrinsic pathway in the hypoproconvertinemia patient after rFVIIa administration in relation to healthy pregnant women. A significant reduction in maximum lysis of the clot after FVII supplementation was observed. Conclusions: The thromboelastometry results showed a significant hypercoagulable state with hypoproconvertinemia. Thrombotic complications after delivery might be prevented by the reduction in rFVIIa guided by thromboelastometry. Thromboelastometry performed on a pregnant woman with factor VII deficiency during the supplementation of rFVIIa in peripartum time might be helpful in order to determine an individual, effective dosage regimen of rFVIIa to ensure full correction of clotting disorders without the tendency to develop thrombosis, but further studies are needed.

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“…To the best of our knowledge, this case is the first ever published on this peculiar clinico-haematological paradox in pregnancy. Despite the fact that several cases with inherited FVII deficiency in pregnancy have been reported in the literature, this has been either in the context of asymptomatic cases that received prophylactic factor replacement to guard against anticipated peripartum haemorrhage, or cases associated with intraoperative bleeding and postpartum haemorrhage 12 14 27–31…”

Section: Discussionmentioning

confidence: 99%

Accidentally discovered high INR in pregnancy unmasks an inherited factor VII (FVII) deficiency that is paradoxically associated with thrombotic tendency

Elkhateb¹,

Mousa

Eldeen

et al. 2021

BMJ Case Rep

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A 32-year-old multiparous obese woman was referred to our center at 37 weeks of twin gestation. She was referred for birth planning following an accidentally discovered high international normalised ratio (INR) in routine preoperative labs. Her history was significant for recurrent pregnancy-associated deep venous thrombosis as well as two early pregnancy losses. Further work-up revealed transaminitis, mild splenomegaly and high lupus anticoagulant titre. A multidisciplinary team of physicians from the high-risk pregnancy, anaesthesiology, haematology, gastroenterology and hepatology departments put a management plan; it culminated into uncomplicated delivery of the patient by repeated caesarian section. The team was also able to figure out the cause of the patient’s high INR that is associated with thrombophilia rather than haemophilia.

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Management of pregnancy in women with factor VII deficiency: A case series

Cited by 21 publications

References 18 publications

Reproductive health and hemostatic issues in women and girls with congenital factor VII deficiency: A systematic review

Reproductive health and hemostatic issues in women and girls with congenital factor VII deficiency: A systematic review

Thromboelastometry as an Ancillary Tool for Evaluation of Coagulation Status after rFVIIa Therapy in a Pregnant Woman with Severe Hypoproconvertinemia—A Case Series and Review of the Literature

Accidentally discovered high INR in pregnancy unmasks an inherited factor VII (FVII) deficiency that is paradoxically associated with thrombotic tendency

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