Abstract
Inherited Factor VII deficiency is an autosomal recessive coagulation disorder with broad range of bleeding manifestations. The association between bleeding and absolute Factor VII level is poor. Usually, the bleeding is associated with FVII levels of less than 1% of the normal value. Factor VII deficiency is associated with prolongation of prothrombin time only with normal activated partial thromboplastin time. Very few pregnant women have been reported with Factor VII deficiency so far in English literature. We, hereby, report 2 cases along with the review of literature of Factor VII deficiency during pregnancy. Our patients were diagnosed to have Factor VII deficiency after deranged coagulogram with Factor VII level of < 1% and 17.1%, respectively, however could be managed by fresh frozen plasma only in first case and fresh frozen plasma and Factor VII concentrate in second case successfully. Coagulation profile is a simple, easily available, affordable, and life-saving investigation to detect this deficiency in pregnancy. Decision regarding replacement therapy should be individualized on a case-to-case basis.
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References
Perry DJ. Factor VII deficiency. Br J Haematol. 2002;118:689–700.
Mannucci PM, Duga S, Peyvandi F. Recessively inherited coagulation disorders. Blood. 2004;104:1243–52.
Pike GN, Bolton Maggs PHB. Factor deficiencies in pregnancy. Hemat Oncol Clin N Am. 2011;25:359–78.
Bolton-Maggs PH, Perry DJ, Chalmers EA, Parapia LA, Wilde JT, Williams MD, Collins PW, Kitchen S, Bolan G, Mumford AD. The rare coagulation disorders – review with guidelines for management from the United Kingdom Haemophilia Centre Doctors’ Organisation. Haemophilia. 2004;10:593–628.
Baumann Kreuziger LM, Morton CT, Reding MT. Is prophylaxis required for delivery in women with factor VII deficiency? Haemophilia. 2013;19:827–32.
Kulkarni AA, Lee CA, Kadir RA. Pregnancy in women with congenital factor VII deficiency. Haemophilia. 2006;12:413–6.
Das D, Ciantar E. Factor VII deficiency in pregnancy and labour: a case report. Obstet Gynecol Int J. 2014;1(1):00003.
Lee YJ, Ju DH, Yi SW, Lee SS, Sohan WS. Successful Management of maternal Factor VII deficiency in a cesarean section. Obstet Gynecol Sci. 2014;57(4):314–7.
Yazicioglu A, Turgal M, Boyraz G, Yucel OS, Tarakan A, Ozyuncu O, Beksac S. Factor VII deficiency during pregnancy. J Turk Soc Obstet Gynecol. 2013;10:114–7.
Pinar HU, Basaran B, Dogan R. Use of low-dose recombinant factor VIIa in a pregnant patient with factor VII deficiency undergoing a minor surgery. J Obstet Anaesth Crit Care. 2015;5:37–8.
Pfrepper C, Siegemund A, Hildebrandt S, Kronberg J, Scholz U, Niederwieser D. Prophylactic treatment of hereditary severe factor VII deficiency in pregnancy. Blood Coagul Fibrinolysis. 2017;28(6):490–2.
Loddo A, Cornacchia S, Cane FL, Barcellona D, Marongiu F, Melis GB, Angioni S, Paoletti AM, Neri M. Prophylaxis of peripartum haemorrhage using recombinant factor VIIa (rfVIIa) in pregnant women with congenital factor VII deficiency: a case report and literature review. Eur J Obstet Gynecol Reprod Biol. 2019;235:77–80.
Hasoon J, Rivers JM. A case of heterozygous factor VII deficiency in pregnancy. J Obstet Gynaecol. 2020;40(7):1025–6.
Lee EJ, Burey L, Abramovitz S, Desancho MT. Management of pregnancy in women with factor VII deficiency: a case series. Haemophilia. 2020;26(4):652–6.
Wildgoose P, Nemerson Y, Hansen LL, Nielsen FE, Glazer S, Hedner U. Measurement of basal levels of factor VIIa in hemophilia A and B patients. Blood. 1992;80:25–8.
Alexander B, Goldstein R, Landwehr G, Cook CD. Congenital SPCA deficiency: a hitherto unrecognized coagulation defect with hemorrhage rectified by serum and serum fractions. J Clin Invest. 1951;30:596–608.
Rizk DE, Castella A, Shaheen H, Deb P. Factor VII deficiency detected in pregnancy: a case report. Am J Perinatol. 1999;16:223–6.
Robertson LE, Wasserstrum N, Banez E, Vasquez M, Sears DA. Hereditary factor VII deficiency in pregnancy: peripartum treatment with factor VII concentrate. Am J Hematol. 1992;40:38–41.
Braun MW, Triplett DA. Case report: Factor VII deficiency in an obstetrical patient. J Indian State Med Assoc. 1979;72:900–2.
Eskandari N, Feldman N, Greenspoon JS. Factor VII deficiency in pregnancy treated with recombinant factor VIIa. Obstet Gynecol. 2002;99:935–7.
Napolitano M, Di Minno MN, Batorova A, Dolce A, Giansily-Blaizot M, Ingerslev J, Schved JF, Auerswald G, Kenet G, Karimi M, Shamsi T, Ruiz de Sáez A, Dolatkhah R, Chuansumrit A, Bertrand MA, Mariani G. Women with congenital factor VII deficiency: clinical phenotype and treatment options from two international studies. Haemophilia. 2016;22(5):752–9.
Di Minno MND, Napolitano M, Dolce A, Mariani G, STER Study Group. Role of clinical and laboratory parameters for treatment choice in patients with inherited FVII deficiency undergoing surgical procedures evidence from the STER registry. Br J Haematol. 2018;180(4):563–70.
Brummel Ziedins K, Rivard GE, Pouliot RL, Butenas S, Gissel M, Parhami-Seren B, et al. Factor VIIa replacement therapy in factor VII deficiency. J Thromb Haemost. 2004;2:1735–44.
Guidelines on the selection and use of therapeutic products to treat haemophilia and other hereditary bleeding disorders. Haemophilia 2003; 9: 1–23.
Farah R, Danaf JA, Braiteh N, Costa JM, Farhat H, Mariani G, Giansily-Blaizot M. Life-threatening bleeding in factor VII deficiency: the role of prenatal diagnosis and primary prophylaxis. Br J Haematol. 2015;16:452–66.
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Minakshi Rohilla—(1) substantial contributions to conception and design, acquisition of data, or analysis and interpretation of data; (2) drafted and revised the article critically for important intellectual content; and (3) final approval of the version to be published.
Rakhi Rai—(1) substantial contributions to conception and design, acquisition of data, or analysis and interpretation of data; (2) drafted and revised the article critically for important intellectual content; and (3) final approval of the version to be published.
Jasmina Ahluwalia—(1) substantial contributions to conception and design, analysis, and interpretation of data; (2) revised the article critically for important intellectual content; and (3) final approval of the version to be published.
Pankaj Malhotra—(1) substantial contributions to conception and design, analysis, and interpretation of data; (2) revised the article critically for important intellectual content; and (3) final approval of the version to be published.
Vanita Jain—(1) substantial contributions to conception and design, analysis, and interpretation of data; (2) revised the article critically for important intellectual content; and (3) final approval of the version to be published.
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Patient Perspective
We were satisfied with the management. We were not aware of the FVII deficiency. The diagnosis will help us in planning future pregnancies. We will be more careful in our day-to-day activities to prevent any injury.
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Rohilla, M., Rai, R., Ahluwalia, J. et al. Factor VII Deficiency and Pregnancy: Case Report and Review of Literature. SN Compr. Clin. Med. 3, 2650–2654 (2021). https://doi.org/10.1007/s42399-021-01037-5
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DOI: https://doi.org/10.1007/s42399-021-01037-5