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Management of Labour and Delivery in a Patient With Acquired Factor VII Deficiency With Inhibitor: A Case Report
CASE REPORT
Management of Labour and Delivery in a Patient With Acquired Factor VII Deficiency With Inhibitor: A Case Report Anca Matei, MD,1 Sean Dolan, MD, FRCPC,2 James Andrews, MD, FRCSC,3 Georges-Étienne Rivard, MD, FRCPC4 1
Department of Obstetrics and Gynecology, Dalhousie University, Halifax NS
2
Department of Hematology, Saint John Regional Hospital, Saint John NB
3
Department of Obstetrics and Gynecology, Saint John Regional Hospital, Saint John NB
4
Department of Hematology, Université de Montréal, Montreal QC
Abstract Background: Acquired factor VII (FVII) deficiency with inhibitor increases the risk of hemorrhage during pregnancy. However, there are no published reports guiding its management in the peripartum period. Case: A 24-year-old woman with inhibitory antibodies to FVII delivered at 34 weeks of gestation. The patient was administered recombinant factor VIIa (rFVIIa) and tranexamic acid. There were no bleedingrelated complications; however, the FVII level was supratherapeutic. The patient returned during a second pregnancy. A reduced dose of rFVIIa was administered. The delivery was complicated by postpartum hemorrhage, which resolved with the addition of uterotonic agents. Conclusion: Recombinant FVIIa and tranexamic acid offer an effective peripartum treatment in women with inhibitory antibody to FVII. Further research should delineate the optimal time of administration.
Résumé Contexte : Le déficit acquis en facteur VII (FVII) chez les patientes qui présentent des anticorps inhibiteurs entraîne une hausse du risque d’hémorragie pendant la grossesse. Toutefois, aucun rapport publié n’en guide la prise en charge pendant la période péripartum. Cas : Une femme de 24 ans présentant des anticorps inhibiteurs en ce qui concerne le FVII a accouché à 34 semaines de gestation. Nous lui avons administré du facteur VIIa recombinant (rFVIIa) et de l’acide tranexamique. Aucune complication associée aux saignements n’a été constatée; toutefois, le niveau de FVII était suprathérapeutique. La patiente nous a de nouveau consultés dans le cadre d’une deuxième grossesse. Une dose moindre de rFVIIa lui alors été administrée. L’accouchement a été compliqué par une hémorragie postpartum, laquelle s’est résorbée à la suite de l’ajout d’agents utérotoniques.
Key Words: Factor VII deficiency, recombinant factor VIIa, pregnancy, postpartum hemorrhage, coagulopathy Competing Interests: None declared. Received on July 2, 2015 Accepted on October 2, 2015 http://dx.doi.org/10.1016/j.jogc.2015.11.002
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Conclusion : L’administration de facteur VIIa recombinant et d’acide tranexamique constitue un traitement péripartum efficace pour les femmes qui présentent des anticorps inhibiteurs en ce qui concerne le FVII. La tenue d’autres recherches s’avère requise pour la détermination du moment optimal pour l’administration de ce traitement. Copyright ª 2016 The Society of Obstetricians and Gynaecologists of Canada/La Société des obstétriciens et gynécologues du Canada. Published by Elsevier Inc. All rights reserved.
J Obstet Gynaecol Can 2016;38(2):160-163
INTRODUCTION
A
cquired factor VII (FVII) deficiency with an inhibitory antibody has been described in only a few case reports.1e5 Management principles reported include FVII replacement and elimination of the inhibitor by apharesis or immunosuppression. The hemostatic level of FVII in the presence of an inhibitor is unknown. In congenital FVII deficiency, a level of 10 to 15 units per decilitre has been proposed,3 but FVII levels have been poorly correlated with clinical manifestations of bleeding.6 Pregnancy poses challenges for management in patients with coagulopathies. In pregnancy, FVII activity increases fourfold in the third trimester.6 With congenital FVII deficiency this response is impaired, but the risk of obstetric hemorrhage does not parallel plasma markers of disease.7 Whether these findings are applicable to patients with the acquired factor deficiency is unknown. We describe here the peripartum management of acquired FVII deficiency due to a known inhibitor antibody to FVII during two consecutive pregnancies in the same patient.
Management of Labour and Delivery in a Patient With Acquired Factor VII Deficiency With Inhibitor: A Case Report
Figure. Factor VII levels at diagnosis and over the course of the first and second pregnancies (normal range 0.51 to 1.54 U/mL). EGA, estimated gestational age; d, days; PP, postpartum; PPD, postpartum day; w, weeks; mo, months
THE CASE
A 24-year-old woman, gravida 3 para 0, presented with threatened preterm labour at 34 weeks of gestation. Her obstetric history included an uncomplicated therapeutic abortion and an early miscarriage. The latter was managed expectantly, and the patient had significant prolonged vaginal bleeding and ecchymoses. Prothrombin time (PT) was prolonged at 49.5 seconds (normal 10.6 to 13.2), PT INR was 4.2 (normal 0.8 to 1.2), and activated partial thromboplastin time was normal. Further evaluation revealed an isolated FVII level of 0.03 U/mL (range 0.51 to 1.54). Mixing studies suggested the presence of an inhibitor. A Bethesda assay also demonstrated the presence of an inhibitor to FVII measuring 5 Bethesda units (normal 0). Screening for lupus anticoagulant and anticardiolipin antibodies was negative. Family and personal histories were noncontributory. It was hypothesized that the patient acquired the inhibitor during a previous pregnancy. Her current pregnancy had been uneventful. In the management plan created by a multidisciplinary team including representatives of obstetrics, maternal-fetal medicine, and hematology, the patient would be administered
recombinant factor VIIa (rFVIIa) at the onset of labour and for at least 24 hours postpartum. Because of the potential risk of fetal bleeding due to transplacental transfer of the inhibitor to the fetus, it was deemed important to avoid fetal scalp sampling, fetal scalp electrodes, and vacuum or forceps delivery. The patient’s coagulation parameters were abnormal, and the behaviour of this rare coagulopathy in labour was unknown; both of these indicated a need to avoid neuraxial anaesthesia.8 An antepartum anaesthesia consultation emphasized concerns about spinal or epidural hematoma, which could cause spinal compression, and the consultant advised against the use of neuraxial analgesia. Following a period of relative uterine quiescence, the patient rapidly progressed through labour. She had vaginal delivery of a healthy infant weighing 2380 g. The patient received rFVIIa, 90 mg/kg, at the onset of the third stage of labour. The placenta was retained, and after 30 minutes of expectant management it was removed manually. No excessive maternal or fetal bleeding was noted. Recombinant FVIIa dosage was repeated every two hours for 24 hours. The patient also received oral
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CASE REPORT
tranexamic acid, 1000 mg every eight hours for 14 days. Her postpartum course was unremarkable. The FVII level immediately postpartum was high (Figure) and PT INR was 0.5, but there was no evidence of thromboembolism. The patient was discharged on the seventh postpartum day. At two weeks postpartum, FVII inhibitor level was 1 Bethesda unit and PT INR 2.3. Six months later, the patient conceived again. Cervical length at 15 weeks of gestation was 5.1 cm. The treatment protocol was adjusted to optimize hemostasis without elevating the risk of thrombosis. The patient presented in labour at 38 weeks’ gestation. When cervical dilatation reached 8 cm, she received rFVIIa 45 mg/kg every two hours for 24 hours. The protocol directed that the dose could be increased if clinically indicated. Intrapartum complications included mild shoulder dystocia and postpartum hemorrhage. The hemorrhage was attributed to uterine atony. Hemostasis was achieved by adding the typical uterotonic agents employed in the management of postpartum hemorrhage due to uterine atony: oxytocin infusion (30 mU per litre of Ringer’s lactate solution), rectal misoprostol (1000 mg), ergotamine (250 mg IM for one dose), and carboprost (250 mg IM for one dose). Her hemoglobin decreased from 104 g/L to 78 g/L and she was transfused two units of packed red blood cells. Tranexamic acid 1000 mg orally three times daily for 10 days was initiated immediately after delivery. There were no thromboembolic events. She delivered a healthy male infant weighing 3640 g. During her intrapartum course, INR remained stable at 1.5. Activated partial thromboplastin time was 27.4 seconds before administration of rFVIIa and 25 seconds after administration (normal 23.0 to 37.0). The change in FVII levels over time is depicted in the Figure. Inhibitor levels were not monitored during her second pregnancy. The infants were not tested for FVII deficiency because they did not show any clinical evidence of bleeding. No other family members were tested. DISCUSSION
We report the first case of acquired FVII deficiency with inhibitor in pregnancy. Treatment consisted of rFVIIa and the antifibrinolytic tranexamic acid. In the first delivery, hemostasis was adequate but the FVII level was elevated. In the second pregnancy a lower dose of rFVIIa was administered. Postpartum hemorrhage did occur and resolved with rFVIIa, tranexamic acid, and uterotonic agents.
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Hemorrhage in a mother with a coagulopathy or her infant can have catastrophic outcomes. There have been no previous reports of peripartum management of acquired FVII deficiency with inhibitor, and we drew dosage information from manufacturer’s recommendations and prior literature on coagulopathies in high-risk settings.9 We treated the patient prophylactically with rFVIIa and used the manufacturer-recommended dose for hemophilia with inhibitor rather than the dose for congenital FVII deficiency, because we hypothesized that an inhibitor poses unique challenges not seen in the congenital form of disease. An unforeseen complication was the timing of the first dose during the patient’s first pregnancy. Because of precipitous progression in labour and because the compound requires reconstitution in the laboratory immediately before being supplied, the first dose was given in the third stage, and not at the onset of labour as planned. Other agents have been successfully used in patients with coagulopathies (packed red blood cells, fresh frozen plasma, prothrombin complex concentrate), but they carry the risk of volume overload, thrombosis, and infection with bloodborne pathogens. Plasma-derived FVII concentrate has also been used,1 but this would have been ineffective in the presence of an inhibitor, as in our case. Intravenous immunoglobulin and immunosuppression with corticosteroids and cyclophosphamide have been used in non-pregnant patients.3 In pregnancy, high doses of glucocorticoids can lead to fetal adrenal suppression and significant maternal side effects, while use of cyclophosphamide should be limited to life-threatening situations when safer options are not available. We believe that rFVIIa was an adequate choice in our case because its synthetic form protects it from inhibition by the patient’s plasma and it carries a low risk of volume overload and infection. Patients must be monitored for thromboembolism; however, this risk in otherwise healthy patients is probably low.9 A major concern in the management of this patient was transplacental transmission of the inhibitory antibody. In women with hemophilia, vertical transmission of an immunoglobulin class G (IgG) inhibitor is possible.10 The FVII inhibitor is also an IgG4 and its vertical transfer may increase the risk of fetal bleeding. This risk should decrease over time as the protein is cleared from the infant’s circulation. Because of this potential risk, invasive fetal procedures should be avoided during labour and delivery. Caesarean section carries a lower risk of fetal intracranial hemorrhage than operative vaginal delivery in unaffected individuals11 and in fetuses with inherited hemophilia;
Management of Labour and Delivery in a Patient With Acquired Factor VII Deficiency With Inhibitor: A Case Report
however, unassisted, spontaneous vaginal delivery offers the lowest risk overall.8 Until more information is available, Caesarean section should be performed if immediate delivery is indicated and an unassisted vaginal delivery is not imminent. Follow-up of these patients should include monitoring of FVII levels and coagulation parameters as clinically indicated. CONCLUSION
This report demonstrates a novel case of peripartum management of a pregnant woman with acquired FVII deficiency with inhibitor. Recombinant FVIIa and tranexamic acid provided adequate hemorrhage prophylaxis in the first pregnancy. In the second, a lower dose of rFVIIa required the addition of uterotonic agents for control of postpartum hemorrhage. There was no clinical evidence of fetal bleeding. ACKNOWLEDGEMENTS
The woman whose story is told in this case report has provided written consent for its publication. The authors wish to thank Louise Bedard, RN, Department of Hematology, Saint John Regional Hospital, Saint John, New Brunswick, and Dr Erica Holloway, Department of Obstetrics and Gynecology, Dr Everett Chalmers Hospital, Fredericton, New Brunswick. The views expressed in the present case report are the researchers’ only and do not express the official positions
of Dalhousie University, Université de Montréal, or Saint John Regional Hospital. REFERENCES 1. Brunod M, Chatot-Henry C, Mehdaoui H, Richer C, Fonteau C. Acquired anti-factor VII (proconvertin) inhibitor: hemorrhage and thrombosis. Thromb Haemost 1998;79:1065e6. 2. de Raucourt E, Dumont MD, Tourani JM, Hubsch JP, Riquet M, Fischer AM. Acquired factor VII deficiency associated with pleural liposarcoma. Blood Coagul Fibrinolysis 1994;5:833e6. 3. Delmer A, Horellou MH, Andreu G, Lecompte T, Rossi F, Kazatchkine MD, et al. Life-threatening intracranial bleeding associated with the presence of an antifactor VII autoantibody. Blood 1989;74:229e32. 4. Kamikubo Y, Miyamoto S, Iwasa A, Ishii M, Okajima K. Purification and characterization of factor VII inhibitor found in a patient with life threatening bleeding. Thromb Haemost 2000;83:60e4. 5. Okajima K, Ishii M. Life-threatening bleeding in a case of autoantibodyinduced factor VII deficiency. Int J Hematol 1999;69:129e32. 6. Eskandari N, Feldman N, Greenspoon JS. Factor VII deficiency in pregnancy treated with recombinant factor VIIa. Obstet Gynecol 2002;99(5 Pt 2):935e7. 7. Kolucki FR Jr, Morris GJ, Thomas LC, Scialla S. Factor VII deficiency in pregnancy and delivery: A case report. Haemophilia 2011;17(6):e1005. 8. Kadir R, Chi C, Bolton-Maggs P. Pregnancy and rare bleeding disorders. Haemophilia 2009;15:990e1005. 9. Franchini M. Recombinant factor VIIa: a review on its clinical use. Int J Hematol 2006;83:126e38. 10. Kotani Y, Shiota M, Umemoto M, Koike E, Tsuritani M, Hoshiai H. Case of acquired hemophilia with factor VIII inhibitor in a mother and newborn. J Obstet Gynaecol Res 2011;37:1102e5. 11. James AH, Hoots K. The optimal mode of delivery for the haemophilia carrier expecting an affected infant is caesarean delivery. Haemophilia 2010;16:420e4.
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Management of Labour and Delivery in a Patient With Acquired Factor VII Deficiency With Inhibitor: A Case Report Anca Matei, MD,1 Sean Dolan, MD, FRCPC,2 James Andrews, MD, FRCSC,3 Georges-Étienne Rivard, MD, FRCPC4 1
Department of Obstetrics and Gynecology, Dalhousie University, Halifax NS
2
Department of Hematology, Saint John Regional Hospital, Saint John NB
3
Department of Obstetrics and Gynecology, Saint John Regional Hospital, Saint John NB
4
Department of Hematology, Université de Montréal, Montreal QC
Abstract Background: Acquired factor VII (FVII) deficiency with inhibitor increases the risk of hemorrhage during pregnancy. However, there are no published reports guiding its management in the peripartum period. Case: A 24-year-old woman with inhibitory antibodies to FVII delivered at 34 weeks of gestation. The patient was administered recombinant factor VIIa (rFVIIa) and tranexamic acid. There were no bleedingrelated complications; however, the FVII level was supratherapeutic. The patient returned during a second pregnancy. A reduced dose of rFVIIa was administered. The delivery was complicated by postpartum hemorrhage, which resolved with the addition of uterotonic agents. Conclusion: Recombinant FVIIa and tranexamic acid offer an effective peripartum treatment in women with inhibitory antibody to FVII. Further research should delineate the optimal time of administration.
Résumé Contexte : Le déficit acquis en facteur VII (FVII) chez les patientes qui présentent des anticorps inhibiteurs entraîne une hausse du risque d’hémorragie pendant la grossesse. Toutefois, aucun rapport publié n’en guide la prise en charge pendant la période péripartum. Cas : Une femme de 24 ans présentant des anticorps inhibiteurs en ce qui concerne le FVII a accouché à 34 semaines de gestation. Nous lui avons administré du facteur VIIa recombinant (rFVIIa) et de l’acide tranexamique. Aucune complication associée aux saignements n’a été constatée; toutefois, le niveau de FVII était suprathérapeutique. La patiente nous a de nouveau consultés dans le cadre d’une deuxième grossesse. Une dose moindre de rFVIIa lui alors été administrée. L’accouchement a été compliqué par une hémorragie postpartum, laquelle s’est résorbée à la suite de l’ajout d’agents utérotoniques.
Key Words: Factor VII deficiency, recombinant factor VIIa, pregnancy, postpartum hemorrhage, coagulopathy Competing Interests: None declared. Received on July 2, 2015 Accepted on October 2, 2015 http://dx.doi.org/10.1016/j.jogc.2015.11.002
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Conclusion : L’administration de facteur VIIa recombinant et d’acide tranexamique constitue un traitement péripartum efficace pour les femmes qui présentent des anticorps inhibiteurs en ce qui concerne le FVII. La tenue d’autres recherches s’avère requise pour la détermination du moment optimal pour l’administration de ce traitement. Copyright ª 2016 The Society of Obstetricians and Gynaecologists of Canada/La Société des obstétriciens et gynécologues du Canada. Published by Elsevier Inc. All rights reserved.
J Obstet Gynaecol Can 2016;38(2):160-163
INTRODUCTION
A
cquired factor VII (FVII) deficiency with an inhibitory antibody has been described in only a few case reports.1e5 Management principles reported include FVII replacement and elimination of the inhibitor by apharesis or immunosuppression. The hemostatic level of FVII in the presence of an inhibitor is unknown. In congenital FVII deficiency, a level of 10 to 15 units per decilitre has been proposed,3 but FVII levels have been poorly correlated with clinical manifestations of bleeding.6 Pregnancy poses challenges for management in patients with coagulopathies. In pregnancy, FVII activity increases fourfold in the third trimester.6 With congenital FVII deficiency this response is impaired, but the risk of obstetric hemorrhage does not parallel plasma markers of disease.7 Whether these findings are applicable to patients with the acquired factor deficiency is unknown. We describe here the peripartum management of acquired FVII deficiency due to a known inhibitor antibody to FVII during two consecutive pregnancies in the same patient.
Management of Labour and Delivery in a Patient With Acquired Factor VII Deficiency With Inhibitor: A Case Report
Figure. Factor VII levels at diagnosis and over the course of the first and second pregnancies (normal range 0.51 to 1.54 U/mL). EGA, estimated gestational age; d, days; PP, postpartum; PPD, postpartum day; w, weeks; mo, months
THE CASE
A 24-year-old woman, gravida 3 para 0, presented with threatened preterm labour at 34 weeks of gestation. Her obstetric history included an uncomplicated therapeutic abortion and an early miscarriage. The latter was managed expectantly, and the patient had significant prolonged vaginal bleeding and ecchymoses. Prothrombin time (PT) was prolonged at 49.5 seconds (normal 10.6 to 13.2), PT INR was 4.2 (normal 0.8 to 1.2), and activated partial thromboplastin time was normal. Further evaluation revealed an isolated FVII level of 0.03 U/mL (range 0.51 to 1.54). Mixing studies suggested the presence of an inhibitor. A Bethesda assay also demonstrated the presence of an inhibitor to FVII measuring 5 Bethesda units (normal 0). Screening for lupus anticoagulant and anticardiolipin antibodies was negative. Family and personal histories were noncontributory. It was hypothesized that the patient acquired the inhibitor during a previous pregnancy. Her current pregnancy had been uneventful. In the management plan created by a multidisciplinary team including representatives of obstetrics, maternal-fetal medicine, and hematology, the patient would be administered
recombinant factor VIIa (rFVIIa) at the onset of labour and for at least 24 hours postpartum. Because of the potential risk of fetal bleeding due to transplacental transfer of the inhibitor to the fetus, it was deemed important to avoid fetal scalp sampling, fetal scalp electrodes, and vacuum or forceps delivery. The patient’s coagulation parameters were abnormal, and the behaviour of this rare coagulopathy in labour was unknown; both of these indicated a need to avoid neuraxial anaesthesia.8 An antepartum anaesthesia consultation emphasized concerns about spinal or epidural hematoma, which could cause spinal compression, and the consultant advised against the use of neuraxial analgesia. Following a period of relative uterine quiescence, the patient rapidly progressed through labour. She had vaginal delivery of a healthy infant weighing 2380 g. The patient received rFVIIa, 90 mg/kg, at the onset of the third stage of labour. The placenta was retained, and after 30 minutes of expectant management it was removed manually. No excessive maternal or fetal bleeding was noted. Recombinant FVIIa dosage was repeated every two hours for 24 hours. The patient also received oral
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CASE REPORT
tranexamic acid, 1000 mg every eight hours for 14 days. Her postpartum course was unremarkable. The FVII level immediately postpartum was high (Figure) and PT INR was 0.5, but there was no evidence of thromboembolism. The patient was discharged on the seventh postpartum day. At two weeks postpartum, FVII inhibitor level was 1 Bethesda unit and PT INR 2.3. Six months later, the patient conceived again. Cervical length at 15 weeks of gestation was 5.1 cm. The treatment protocol was adjusted to optimize hemostasis without elevating the risk of thrombosis. The patient presented in labour at 38 weeks’ gestation. When cervical dilatation reached 8 cm, she received rFVIIa 45 mg/kg every two hours for 24 hours. The protocol directed that the dose could be increased if clinically indicated. Intrapartum complications included mild shoulder dystocia and postpartum hemorrhage. The hemorrhage was attributed to uterine atony. Hemostasis was achieved by adding the typical uterotonic agents employed in the management of postpartum hemorrhage due to uterine atony: oxytocin infusion (30 mU per litre of Ringer’s lactate solution), rectal misoprostol (1000 mg), ergotamine (250 mg IM for one dose), and carboprost (250 mg IM for one dose). Her hemoglobin decreased from 104 g/L to 78 g/L and she was transfused two units of packed red blood cells. Tranexamic acid 1000 mg orally three times daily for 10 days was initiated immediately after delivery. There were no thromboembolic events. She delivered a healthy male infant weighing 3640 g. During her intrapartum course, INR remained stable at 1.5. Activated partial thromboplastin time was 27.4 seconds before administration of rFVIIa and 25 seconds after administration (normal 23.0 to 37.0). The change in FVII levels over time is depicted in the Figure. Inhibitor levels were not monitored during her second pregnancy. The infants were not tested for FVII deficiency because they did not show any clinical evidence of bleeding. No other family members were tested. DISCUSSION
We report the first case of acquired FVII deficiency with inhibitor in pregnancy. Treatment consisted of rFVIIa and the antifibrinolytic tranexamic acid. In the first delivery, hemostasis was adequate but the FVII level was elevated. In the second pregnancy a lower dose of rFVIIa was administered. Postpartum hemorrhage did occur and resolved with rFVIIa, tranexamic acid, and uterotonic agents.
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Hemorrhage in a mother with a coagulopathy or her infant can have catastrophic outcomes. There have been no previous reports of peripartum management of acquired FVII deficiency with inhibitor, and we drew dosage information from manufacturer’s recommendations and prior literature on coagulopathies in high-risk settings.9 We treated the patient prophylactically with rFVIIa and used the manufacturer-recommended dose for hemophilia with inhibitor rather than the dose for congenital FVII deficiency, because we hypothesized that an inhibitor poses unique challenges not seen in the congenital form of disease. An unforeseen complication was the timing of the first dose during the patient’s first pregnancy. Because of precipitous progression in labour and because the compound requires reconstitution in the laboratory immediately before being supplied, the first dose was given in the third stage, and not at the onset of labour as planned. Other agents have been successfully used in patients with coagulopathies (packed red blood cells, fresh frozen plasma, prothrombin complex concentrate), but they carry the risk of volume overload, thrombosis, and infection with bloodborne pathogens. Plasma-derived FVII concentrate has also been used,1 but this would have been ineffective in the presence of an inhibitor, as in our case. Intravenous immunoglobulin and immunosuppression with corticosteroids and cyclophosphamide have been used in non-pregnant patients.3 In pregnancy, high doses of glucocorticoids can lead to fetal adrenal suppression and significant maternal side effects, while use of cyclophosphamide should be limited to life-threatening situations when safer options are not available. We believe that rFVIIa was an adequate choice in our case because its synthetic form protects it from inhibition by the patient’s plasma and it carries a low risk of volume overload and infection. Patients must be monitored for thromboembolism; however, this risk in otherwise healthy patients is probably low.9 A major concern in the management of this patient was transplacental transmission of the inhibitory antibody. In women with hemophilia, vertical transmission of an immunoglobulin class G (IgG) inhibitor is possible.10 The FVII inhibitor is also an IgG4 and its vertical transfer may increase the risk of fetal bleeding. This risk should decrease over time as the protein is cleared from the infant’s circulation. Because of this potential risk, invasive fetal procedures should be avoided during labour and delivery. Caesarean section carries a lower risk of fetal intracranial hemorrhage than operative vaginal delivery in unaffected individuals11 and in fetuses with inherited hemophilia;
Management of Labour and Delivery in a Patient With Acquired Factor VII Deficiency With Inhibitor: A Case Report
however, unassisted, spontaneous vaginal delivery offers the lowest risk overall.8 Until more information is available, Caesarean section should be performed if immediate delivery is indicated and an unassisted vaginal delivery is not imminent. Follow-up of these patients should include monitoring of FVII levels and coagulation parameters as clinically indicated. CONCLUSION
This report demonstrates a novel case of peripartum management of a pregnant woman with acquired FVII deficiency with inhibitor. Recombinant FVIIa and tranexamic acid provided adequate hemorrhage prophylaxis in the first pregnancy. In the second, a lower dose of rFVIIa required the addition of uterotonic agents for control of postpartum hemorrhage. There was no clinical evidence of fetal bleeding. ACKNOWLEDGEMENTS
The woman whose story is told in this case report has provided written consent for its publication. The authors wish to thank Louise Bedard, RN, Department of Hematology, Saint John Regional Hospital, Saint John, New Brunswick, and Dr Erica Holloway, Department of Obstetrics and Gynecology, Dr Everett Chalmers Hospital, Fredericton, New Brunswick. The views expressed in the present case report are the researchers’ only and do not express the official positions
of Dalhousie University, Université de Montréal, or Saint John Regional Hospital. REFERENCES 1. Brunod M, Chatot-Henry C, Mehdaoui H, Richer C, Fonteau C. Acquired anti-factor VII (proconvertin) inhibitor: hemorrhage and thrombosis. Thromb Haemost 1998;79:1065e6. 2. de Raucourt E, Dumont MD, Tourani JM, Hubsch JP, Riquet M, Fischer AM. Acquired factor VII deficiency associated with pleural liposarcoma. Blood Coagul Fibrinolysis 1994;5:833e6. 3. Delmer A, Horellou MH, Andreu G, Lecompte T, Rossi F, Kazatchkine MD, et al. Life-threatening intracranial bleeding associated with the presence of an antifactor VII autoantibody. Blood 1989;74:229e32. 4. Kamikubo Y, Miyamoto S, Iwasa A, Ishii M, Okajima K. Purification and characterization of factor VII inhibitor found in a patient with life threatening bleeding. Thromb Haemost 2000;83:60e4. 5. Okajima K, Ishii M. Life-threatening bleeding in a case of autoantibodyinduced factor VII deficiency. Int J Hematol 1999;69:129e32. 6. Eskandari N, Feldman N, Greenspoon JS. Factor VII deficiency in pregnancy treated with recombinant factor VIIa. Obstet Gynecol 2002;99(5 Pt 2):935e7. 7. Kolucki FR Jr, Morris GJ, Thomas LC, Scialla S. Factor VII deficiency in pregnancy and delivery: A case report. Haemophilia 2011;17(6):e1005. 8. Kadir R, Chi C, Bolton-Maggs P. Pregnancy and rare bleeding disorders. Haemophilia 2009;15:990e1005. 9. Franchini M. Recombinant factor VIIa: a review on its clinical use. Int J Hematol 2006;83:126e38. 10. Kotani Y, Shiota M, Umemoto M, Koike E, Tsuritani M, Hoshiai H. Case of acquired hemophilia with factor VIII inhibitor in a mother and newborn. J Obstet Gynaecol Res 2011;37:1102e5. 11. James AH, Hoots K. The optimal mode of delivery for the haemophilia carrier expecting an affected infant is caesarean delivery. Haemophilia 2010;16:420e4.
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