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Sachdeva M, Kaur A, Aggarwal N, Ahluwalia J, Vohra S. Feto-Maternal Outcomes in Pregnancy With Factor VII Deficiency in a Tertiary Care Institution. Cureus 2024; 16:e64079. [PMID: 39114246 PMCID: PMC11305144 DOI: 10.7759/cureus.64079] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 07/08/2024] [Indexed: 08/10/2024] Open
Abstract
This paper aims to study antepartum and postpartum bleeding manifestations of patients with factor VII (FVII) deficiency, their management, and feto-maternal outcomes, to establish danger signs and management protocols. We describe a case series of nine pregnancies in four patients with FVII deficiency diagnosed at a tertiary care referral center in India between 2012 and 2023. Out of nine pregnancies, six had cesarean deliveries, two had vaginal deliveries, and one had dilatation and curettage for unwanted pregnancies. One out of nine pregnancies (11.11%) with an unknown FVII deficiency had antepartum hemorrhage (abruption) necessitating multiple transfusions, ICU stay, and neonatal loss. Three patients with no prior history of obstetric hemorrhage were diagnosed with severe deficiencies and received prophylactic recombinant FVII preoperatively, averting the potential loss of lives. In patients with no history of bleeding, no hemorrhage was reported with or without prophylaxis while 33.33% of hemorrhage was reported in patients with a history of bleeding. Factors like the history of bleeding, FVII levels, mode of delivery, and other risk factors for hemorrhage should all be considered to predict the risk of bleeding in delivery. Cesarean is a surgical procedure, and prophylactic use of recombinant FVII concentrate (rFVIIa) should be considered.
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Affiliation(s)
- Mohini Sachdeva
- Obstetrics and Gynecology, Postgraduate Institute of Medical Education and Research, Chandigarh, IND
| | - Amanjot Kaur
- Obstetrics and Gynecology, Postgraduate Institute of Medical Education and Research, Chandigarh, IND
| | - Neelam Aggarwal
- Obstetrics and Gynecology, Postgraduate Institute of Medical Education and Research, Chandigarh, IND
| | - Jasmina Ahluwalia
- Hematology, Postgraduate Institute of Medical Education and Research, Chandigarh, IND
| | - Simran Vohra
- Obstetrics and Gynecology, Postgraduate Institute of Medical Education and Research, Chandigarh, IND
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2
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Hajjar R, Hatoum I, Krounbi A, Chahine R, Jalloul R, Ramadan MK. Pregnancy in a woman with congenital F-VII deficiency: a brief review of recent literature and case report. Hematol Transfus Cell Ther 2023; 45:394-398. [PMID: 34565715 PMCID: PMC10499559 DOI: 10.1016/j.htct.2021.07.003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/02/2021] [Accepted: 07/10/2021] [Indexed: 11/29/2022] Open
Affiliation(s)
- Rima Hajjar
- American University in Beirut-Medical Center, Department of Obstetrics and Gynecology, Beirut, Lebanon
| | - Inaam Hatoum
- Rafik Hariri University Hospital, Beirut, Lebanon
| | | | - Rabih Chahine
- American University in Beirut-Medical Center, Department of Obstetrics and Gynecology, Beirut, Lebanon; Rafik Hariri University Hospital, Beirut, Lebanon
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Obore N, Jin W, Huiqin Q, Wei Q, Yan H, Hong Y. Pregnant Women with Severe Factor VII Deficiency Undergoing Cesarean Section Managed with a Short-Term Regimen of Recombinant Factor VIIa. MATERNAL-FETAL MEDICINE 2023; 5:195-198. [PMID: 40416855 PMCID: PMC12096402 DOI: 10.1097/fm9.0000000000000194] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/11/2022] [Accepted: 04/28/2023] [Indexed: 05/27/2025] Open
Affiliation(s)
- Nathan Obore
- Department of Obstetrics and Gynecology, Zhongda Hospital, Southeast University, Nanjing 210009, China
| | - Wan Jin
- Department of Obstetrics and Gynecology, Zhongda Hospital, Southeast University, Nanjing 210009, China
| | - Qian Huiqin
- Department of Obstetrics and Gynecology, Zhongda Hospital, Southeast University, Nanjing 210009, China
| | - Qian Wei
- Department of Obstetrics and Gynecology, Zhongda Hospital, Southeast University, Nanjing 210009, China
| | - Hu Yan
- Department of Obstetrics and Gynecology, Zhongda Hospital, Southeast University, Nanjing 210009, China
| | - Yu Hong
- Department of Obstetrics and Gynecology, Zhongda Hospital, Southeast University, Nanjing 210009, China
- Guidance Center of Jiangsu Maternal Critical Care, Nanjing 210009, China
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Abdul‐Kadir R, Gomez K. Reproductive health and hemostatic issues in women and girls with congenital factor VII deficiency: A systematic review. J Thromb Haemost 2022; 20:2758-2772. [PMID: 36073900 PMCID: PMC9828586 DOI: 10.1111/jth.15872] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/17/2022] [Revised: 08/30/2022] [Accepted: 09/06/2022] [Indexed: 01/13/2023]
Abstract
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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Affiliation(s)
- Rezan Abdul‐Kadir
- Katharine Dormandy Haemophilia Centre and Department of Obstetrics & GynaecologyRoyal Free HospitalLondonUK
| | - Keith Gomez
- Haemophilia Centre and Thrombosis UnitRoyal Free London NHS Foundation TrustLondonUK
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Krzyżanowski A, Gęca T, Sokołowska B, Kwiatek M, Miturski A, Stupak A, Terlecki P, Paluszkiewicz P, Kwaśniewska A. 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. INTERNATIONAL JOURNAL OF ENVIRONMENTAL RESEARCH AND PUBLIC HEALTH 2022; 19:10918. [PMID: 36078653 PMCID: PMC9518547 DOI: 10.3390/ijerph191710918] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Subscribe] [Scholar Register] [Received: 07/09/2022] [Revised: 08/20/2022] [Accepted: 08/30/2022] [Indexed: 06/15/2023]
Abstract
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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Affiliation(s)
- Arkadiusz Krzyżanowski
- Chair and Department of Obstetrics and Pathology of Pregnancy, Medical University of Lublin, 20-081 Lublin, Poland
| | - Tomasz Gęca
- Chair and Department of Obstetrics and Pathology of Pregnancy, Medical University of Lublin, 20-081 Lublin, Poland
| | - Bożena Sokołowska
- Chair and Department of Haematooncology and Bone Marrow Transplantation, Medical University of Lublin, 20-059 Lublin, Poland
| | - Maciej Kwiatek
- Chair and Department of Obstetrics and Pathology of Pregnancy, Medical University of Lublin, 20-081 Lublin, Poland
| | - Andrzej Miturski
- Chair and Department of Obstetrics and Pathology of Pregnancy, Medical University of Lublin, 20-081 Lublin, Poland
| | - Aleksandra Stupak
- Chair and Department of Obstetrics and Pathology of Pregnancy, Medical University of Lublin, 20-081 Lublin, Poland
| | - Piotr Terlecki
- Chair and Department of Vascular Surgery and Angiology, Medical University of Lublin, 20-081 Lublin, Poland
| | - Piotr Paluszkiewicz
- Department of General, Oncological and Metabolic Surgery, Institute of Haematology and Transfusion Medicine, 14 I. Gandhi Str., 02-776 Warsaw, Poland
| | - Anna Kwaśniewska
- Chair and Department of Obstetrics and Pathology of Pregnancy, Medical University of Lublin, 20-081 Lublin, Poland
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Bollici Martínez J, Valiente Álvarez JL, Pérez Castillejo S, López Pérez R, Aguilar Franco C, Del Villar Sordo V. Epidural analgesia in pregnant with deficit of factor VII. REVISTA ESPANOLA DE ANESTESIOLOGIA Y REANIMACION 2022; 69:242-244. [PMID: 35534386 DOI: 10.1016/j.redare.2020.12.007] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 02/17/2020] [Accepted: 12/27/2020] [Indexed: 06/14/2023]
Abstract
The congenital deficit of FVII of coagulation it's an anomaly of genetic transmission autosomal recessive type, it can occur with clinical manifestations like hematomas and spontaneous bleeding or not. The normal levels of FVII it's found between 70%-130% of the laboratory reference value. For unknown reasons there is a poor correlation between levels of FVII and bleeding risk. During pregnancy coagulation can be significantly altered, there is a no clear consensus and a very few information about how to act during labor in a patient with a FVII deficit. The case of a 35-year-old patient with 35 weeks of gestation and congenital deficit of the coagulation FVII (36%) is presented, epidural analgesia is performed during labor previously administering activated recombinant FVII (rFVIIa) without complications (Spinal hematoma, postpartum bleeding, thrombosis).
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Affiliation(s)
- J Bollici Martínez
- Servicio de Anestesiología y Reanimación, Complejo Asistencial de Soria, Soria, Spain.
| | - J L Valiente Álvarez
- Servicio de Anestesiología y Reanimación, Complejo Asistencial de Soria, Soria, Spain
| | - S Pérez Castillejo
- Servicio de Anestesiología y Reanimación, Complejo Asistencial de Soria, Soria, Spain
| | - R López Pérez
- Servicio de Anestesiología y Reanimación, Complejo Asistencial de Soria, Soria, Spain
| | - C Aguilar Franco
- Servicio de Hematología, Complejo Asistencial de Soria, Soria, Spain
| | - V Del Villar Sordo
- Servicio de Medicina Interna y Unidad de Investigación, Complejo Asistencial de Soria, Soria, Spain
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Yang Y, Zeng YC, Rumende P, Wang CG, Chen Y. Diagnosis and treatment discussion of congenital factor VII deficiency in pregnancy: A case report. World J Clin Cases 2021; 9:6091-6101. [PMID: 34368331 PMCID: PMC8316952 DOI: 10.12998/wjcc.v9.i21.6091] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/22/2021] [Revised: 05/09/2021] [Accepted: 05/26/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Congenital factor VII deficiency (FVIID) is a rare autosomal recessive genetic disorder. The clinical manifestations of this deficiency vary greatly. Predicting the risk of bleeding during and after childbirth of pregnant women with congenital FVIID is difficult. Recombinant factor VIIa is the most common replacement therapy for FVIID. However, no unified diagnosis and treatment plan for pregnant women with congenital FVIID has been established.
CASE SUMMARY We report the clinical history of a pregnant woman who was considered to have congenital FVIID. Recombinant factor VIIa was prophylactically administered to the pregnant woman at the time of cervical fully opening. She successfully delivered a live infant without any complications, such as postpartum hemorrhage, neonatal abnormalities, and so on.
CONCLUSION Prophylaxis of recombinant factor VIIa during delivery can effectively reduce the incidence of postpartum hemorrhage among pregnant women with congenital FVIID associated with a high risk of bleeding.
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Affiliation(s)
- Ying Yang
- Department of Obstetrics, The First Affiliated Hospital of Guangxi Medical University, Nanning 530021, Guangxi Zhuang Autonomous Region, China
| | - Ya-Chang Zeng
- Department of Obstetrics, The First Affiliated Hospital of Guangxi Medical University, Nanning 530021, Guangxi Zhuang Autonomous Region, China
| | - Pingkan Rumende
- Department of Obstetrics, The First Affiliated Hospital of Guangxi Medical University, Nanning 530021, Guangxi Zhuang Autonomous Region, China
| | - Chen-Guang Wang
- Department of Obstetrics, The First Affiliated Hospital of Guangxi Medical University, Nanning 530021, Guangxi Zhuang Autonomous Region, China
| | - Yue Chen
- Department of Obstetrics, The First Affiliated Hospital of Guangxi Medical University, Nanning 530021, Guangxi Zhuang Autonomous Region, China
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8
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Bollici Martínez J, Valiente Álvarez JL, Pérez Castillejo S, López Pérez R, Aguilar Franco C, Del Villar Sordo V. Epidural analgesia in pregnant with deficit of factor VII. REVISTA ESPANOLA DE ANESTESIOLOGIA Y REANIMACION 2021; 69:S0034-9356(21)00054-2. [PMID: 33726919 DOI: 10.1016/j.redar.2020.12.010] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 02/17/2020] [Revised: 12/05/2020] [Accepted: 12/27/2020] [Indexed: 06/12/2023]
Abstract
The congenital deficit of FVII of coagulation it's an anomaly of genetic transmission autosomal recessive type, it can occur with clinical manifestations like hematomas and spontaneous bleeding or not. The normal levels of FVII it's found between 70%-130% of the laboratory reference value. For unknown reasons there is a poor correlation between levels of FVII and bleeding risk. During pregnancy coagulation can be significantly altered, there is a no clear consensus and a very few information about how to act during labor in a patient with a FVII deficit. The case of a 35-year-old patient with 35 weeks of gestation and congenital deficit of the coagulation FVII (36%) is presented, epidural analgesia is performed during labor previously administering activated recombinant FVII (rFVIIa) without complications (Spinal hematoma, postpartum bleeding, thrombosis).
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Affiliation(s)
- J Bollici Martínez
- Servicio de Anestesiología y Reanimación, Complejo Asistencial de Soria, Soria, España.
| | - J L Valiente Álvarez
- Servicio de Anestesiología y Reanimación, Complejo Asistencial de Soria, Soria, España
| | - S Pérez Castillejo
- Servicio de Anestesiología y Reanimación, Complejo Asistencial de Soria, Soria, España
| | - R López Pérez
- Servicio de Anestesiología y Reanimación, Complejo Asistencial de Soria, Soria, España
| | - C Aguilar Franco
- Servicio de Hematología, Complejo Asistencial de Soria, Soria, España
| | - V Del Villar Sordo
- Servicio de Medicina Interna y Unidad de Investigación, Complejo Asistencial de Soria, Soria, España
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Peltier S, Kellum A, Brewer J, Duncan A, Cooper DL, Saad H. Psychosocial Impact and Disease Management in Patients with Congenital Factor VII Deficiency. J Blood Med 2020; 11:297-303. [PMID: 32982528 PMCID: PMC7494225 DOI: 10.2147/jbm.s259909] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/25/2020] [Accepted: 08/18/2020] [Indexed: 11/23/2022] Open
Abstract
Purpose Congenital factor VII (FVII) deficiency is a rare bleeding disorder of variable phenotype with predominantly mucocutaneous bleeding. The aim of this study was to identify the burden of FVII deficiency on patients and caregivers through a better understanding of the management and psychosocial impact of this disease. Materials and Methods A rare disease specialty recruiter from Comprehensive Health Education Services recruited participants for this online survey, which was conducted from January 31 to March 12, 2019. A moderator-assisted questionnaire was used to collect data on demographics, diagnosis, treatment, and psychosocial impact. Results Of the 45 respondents (25 patients and 20 caregivers), the majority were female (56%). Respondents reported a wide variety of initial bleeding symptoms, including bruising (58%), epistaxis (56%), and menorrhagia (36% of females). Because symptoms varied between individuals and were not always severe, diagnosis was often delayed. Mean time to obtain a diagnosis was 6.5 years and mean age at first diagnosis was 12.9 years. One-quarter (24%) of the respondents reported more than 100 bleeds of any severity over the previous year. When treating bleeds, 44% of patients reported using antifibrinolytics, and 42% reported using recombinant activated factor VII. Almost 31% of respondents reported missing schooldays as children, and 16% reported losing or resigning from a job in adulthood as a direct result of their disease. Notably, 29% of caregivers and 10% of their partners had also experienced issues with employment. Forty percent of respondents reported not participating in contact sports during childhood, and 22% continued to avoid contact sports in adulthood. Conclusion Overall, FVII deficiency has a substantial psychosocial impact, but most patients are satisfied with their disease management and are optimistic about their future. Patients desire additional educational, social, and financial support.
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Affiliation(s)
- Skye Peltier
- Center for Bleeding and Clotting Disorders, University of Minnesota Medical Center - Fairview, Minneapolis, MN, USA
| | - Angela Kellum
- Louisiana Center for Bleeding and Clotting Disorders, Tulane University, New Orleans, LA, USA
| | - Janet Brewer
- Comprehensive Health Education Services, Hanson, MA, USA
| | - Alexander Duncan
- Department of Pathology & Laboratory Medicine, Emory University School of Medicine, Atlanta, GA, USA
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Abstract
Factor VII (FVII) deficiency is the most common of the Rare Inherited Coagulation Disorders. The inheritance is autosomal recessive but there is variable penetrance. Overall there is poor correlation between the FVII level and the bleeding phenotype. Heterozygotes may have significant bleeding and severe homozygotes, or compound heterozygotes can be asymptomatic. Typically, homozygotes have FVII levels <10% and heterozygotes have levels above that. In most cases bleeding is uncommon with FVII levels>10-20%. A personal and family history is essential to determine the bleeding risk and to plan for surgical and obstetrical prophylaxis. Severe bleeding complications including central nervous system bleeding, gastrointestinal system bleeding and bleeding into the joints occurs in 10-15% of FVII deficient patients. Mucocutaneous bleeding is a common symptom but 30% of patients are asymptomatic. Fifty to 69% of women have heavy menstrual bleeding. Due to the limited number of publications regarding this rare disorder there are no consensus guidelines. There is registry data which has led to the best recommendations for treatment of bleeding episodes, initiation of long-term prophylaxis in addition to surgical plus ante and peripartum prophylaxis. Recombinant FVII concentrate is the best replacement therapy and a review of treatment and prophylaxis dosing is discussed.
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Affiliation(s)
- K Sue Robinson
- Division of Hematology, Dalhousie University, Queen Elizabeth II Health Sciences Centre, Department of Medicine, Rm. 416, 4th Floor Bethune Bldg, 1276 South Park St., Halifax, NS, B3H 2Y9, Canada.
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