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

Elkhateb, Islam Tarek; Mousa, Abdalla; Eldeen, Riham Mohye; Soliman, Yssra

doi:10.1136/bcr-2020-237781

Cited by 5 publications

(23 citation statements)

References 31 publications

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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: 99%

“…Three caesarean deliveries were planned; one to reduce risk of human immunodeficiency virus transmission to the fetus, 51 one due to prior maternal intracranial hemorrhage, 53 and one at maternal request 53 . The rationale for the remaining eight caesarean deliveries was not reported 12,16,19,52,53 …”

Section: Resultsmentioning

confidence: 99%

“… 10 , 11 , 12 , 13 , 14 One woman assessed for consanguinity had non‐severe FVII deficiency; 13 the other six had severe deficiency, including all three women for whom consanguinity was recorded. 10 , 11 , 12 , 14 …”

Section: Resultsmentioning

confidence: 99%

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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: 99%

Section: Resultsmentioning

confidence: 99%

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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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“…In fact, factor VII seems not to play an important role in the warfarin's antithrombotic effect, because venous thrombosis still can be formed in patients with congenital factor VII deficiency from a case report. 11 Blood coagulation cascade is composed of three phases: initiation, amplification, and propagation. The phase of amplification is important for thrombosis.…”

Section: Discussionmentioning

confidence: 99%

The Comparison of Therapeutic Efficacy Between Dabigatran Versus Warfarin in Patients With Nonvalvular Atrial Fibrillation

Zhang¹,

Xia

Zhang

et al. 2021

Clin Appl Thromb Hemost

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Background Novel oral anticoagulants and warfarin are widely used for stroke prevention in patients with atrial fibrillation. The anticoagulation status of patients receiving warfarin or rivaroxaban has been studied. In this study, we aimed to evaluate the effect of dabigatran and warfarin on preventing thrombin generation (TG). Methods This retrospective study enrolled 237 nonvalvular atrial fibrillation (NVAF) subjects treated with 110 mg dabigatran etexilate twice daily and 224 NVAF patients received adjusted-dose warfarin (international normalized ratio [INR] of 2 to 3)). Coagulation assays, prothrombin fragment 1 + 2 (F1+2), calibrated automated thrombogram, and thrombin–antithrombin complex (TAT) were detected at the steady state. Results Activated partial thromboplastin time (APTT), antithrombin III activity, fibrinogen, and lag time showed no difference between the two groups. Compared to the dabigatran group, prothrombin time and INR values were higher in the warfarin group (all P < .001). Thrombin time, endogenous thrombin potential, peak TG (Cmax), F1+2, and TAT were lower in the warfarin group. The inhibition of TG was still stronger in the warfarin group when the patients were divided into subgroups. Conclusion Conventional coagulation assays are suboptimal for assessing the coagulation status of dabigatran. TG could be used as supplementary assays to evaluate the anticoagulation effect of oral anticoagulants. Our results suggest that warfarin may inhibit TG more aggressively than dabigatran in patients regardless of age and kidney function.

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“…El déficit de factor VII, que es una enfermedad autosómica recesiva poco común y tiene una incidencia estimada de 1 en 500.000, las manifestaciones clínicas del déficit de factor VII son muy variables, desde individuos asintomáticos hasta alteraciones sanguíneas severas y potencialmente mortales [3], [4].…”

Section: Introductionunclassified

Caso clínico: beta talasemia y deficiencia de factor VII en embarazo

G.¹,

J.²,

Sánchez³

et al. 2022

Rev. chil. anest.

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

Cited by 5 publications

References 31 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

The Comparison of Therapeutic Efficacy Between Dabigatran Versus Warfarin in Patients With Nonvalvular Atrial Fibrillation

Caso clínico: beta talasemia y deficiencia de factor VII en embarazo

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