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FACTOR XI DEFICIENCY IN PREGNANCY: THREE CASE REPORTS
Author(s): ,
Alessandra Poz
Affiliations:
Department of medical and biological sciences (DSMB),University of Udine,Udine,Italy
,
Marina Turello
Affiliations:
Center for Hemorragic and Thrombotic Diseases,University Hospital of Udine,Udine,Italy
,
Antonella Bertone
Affiliations:
Center for Hemorragic and Thrombotic Diseases,University Hospital of Udine,Udine,Italy
Giovanni Barillari
Affiliations:
Center for Hemorragic and Thrombotic Diseases,University Hospital of Udine,Udine,Italy
(Abstract release date: 05/19/16) EHA Library. Barillari G. 06/09/16; 132552; E1003
Dr. Giovanni Barillari
Dr. Giovanni Barillari
Contributions
Abstract
Abstract: E1003

Type: Eposter Presentation

Background
Management of pregnant women with factor XI deficiency (F11D) poses a challenge to the clinician because of both the variabile uterine bleeding during labor and the risk associated with factor replacement. Hemoleven® is a factor eleven (FXI) concentrate processed by deep filtration and ion exchange chtomatography, with double viral inactivation performed using solvent detergent and nanofiltration. It represents a good treatment for protecting against bleeding, but the potential risk of thrombosis shoul be considered.

Aims
We present the cases of three women with F11D and a history of bleedings, where the efficacy and safety of Hemoleven® treatment was assessed during pregnancy and labor.

Methods
During labor, the drug was administered together with tranexamic acid.

Results
In case 1 the woman was diagnosed as F11D when she was 10, with a plasma FXI level less than 1%  and an omozygote phenotype. The 34 year old primagravidae had an uneventful pregnancy, and she was admitted at 39+1 weeks of gestation. When cutting the umbelical cord, antihaemorragic prophylaxis was performed with 1 g of tranexamic acid, intravenously injected, and 1000 UI of Hemoleven. 1 g of tranexamic acid was then administered every 8 hours, from the first to the eighth day post-partum, while 1000 UI of Hemoleven were given during the second and the fifth day post-partum.In case 2, a 26 year old primagravidae had an uneventful pregnancy, but she was admitted at 38+4 weeks of gestation due to a very big condylomatosis. She was diagnosed as F11D at the age of 17, with a plasma FXI level of 42% and an heterozygote phenotype. Despite FXI level, she had an hemorragic phenotype. Caesaren section was planned and performed at the day of admission, in general anesthesia. 30 minutes before the delivery started, 1000 UI of Hemoleven were infused, and 1 g of tranexamic acid was administered intravenously ad the beginning of the procedure. After caesarean section, 1 g of tranexamic acid was given every 8 hour till the seventh day.In case 3, a 36 year old woman had an uneventful second pregnancy, and she was admitted at 39 weeks of gestation. She was diagnosed as F11D at the age of 31, with a plasma FXI level of 30%. Vaginal delivery was induced with oxitocin. After umbelical cord excision, 1000 UI of Hemoleven were infused and 1 g of tranexamic acid was given intravenously. Subsequently, 1 g of tranexamic acid was then administered every 8 hours, from the first to the seventh day post-partum.

Conclusion
Neither post-partum haemorragy nor thrombotic events were evident in all the three cases, thus confirming safety and efficacy of clotting factor eleven concentrate. Hemoleven® dose and duration of treatmen was alway carefully decided before delivery, with a management plan shared between haemostasis experts, obstetrics, and the patients. Considering our experience, the benefit/risk ratio of Hemoleven® should be weighted and management tailored to each individual patient.

Session topic: E-poster

Keyword(s): Bleeding disorder, Factor Xia, Pregnancy
Abstract: E1003

Type: Eposter Presentation

Background
Management of pregnant women with factor XI deficiency (F11D) poses a challenge to the clinician because of both the variabile uterine bleeding during labor and the risk associated with factor replacement. Hemoleven® is a factor eleven (FXI) concentrate processed by deep filtration and ion exchange chtomatography, with double viral inactivation performed using solvent detergent and nanofiltration. It represents a good treatment for protecting against bleeding, but the potential risk of thrombosis shoul be considered.

Aims
We present the cases of three women with F11D and a history of bleedings, where the efficacy and safety of Hemoleven® treatment was assessed during pregnancy and labor.

Methods
During labor, the drug was administered together with tranexamic acid.

Results
In case 1 the woman was diagnosed as F11D when she was 10, with a plasma FXI level less than 1%  and an omozygote phenotype. The 34 year old primagravidae had an uneventful pregnancy, and she was admitted at 39+1 weeks of gestation. When cutting the umbelical cord, antihaemorragic prophylaxis was performed with 1 g of tranexamic acid, intravenously injected, and 1000 UI of Hemoleven. 1 g of tranexamic acid was then administered every 8 hours, from the first to the eighth day post-partum, while 1000 UI of Hemoleven were given during the second and the fifth day post-partum.In case 2, a 26 year old primagravidae had an uneventful pregnancy, but she was admitted at 38+4 weeks of gestation due to a very big condylomatosis. She was diagnosed as F11D at the age of 17, with a plasma FXI level of 42% and an heterozygote phenotype. Despite FXI level, she had an hemorragic phenotype. Caesaren section was planned and performed at the day of admission, in general anesthesia. 30 minutes before the delivery started, 1000 UI of Hemoleven were infused, and 1 g of tranexamic acid was administered intravenously ad the beginning of the procedure. After caesarean section, 1 g of tranexamic acid was given every 8 hour till the seventh day.In case 3, a 36 year old woman had an uneventful second pregnancy, and she was admitted at 39 weeks of gestation. She was diagnosed as F11D at the age of 31, with a plasma FXI level of 30%. Vaginal delivery was induced with oxitocin. After umbelical cord excision, 1000 UI of Hemoleven were infused and 1 g of tranexamic acid was given intravenously. Subsequently, 1 g of tranexamic acid was then administered every 8 hours, from the first to the seventh day post-partum.

Conclusion
Neither post-partum haemorragy nor thrombotic events were evident in all the three cases, thus confirming safety and efficacy of clotting factor eleven concentrate. Hemoleven® dose and duration of treatmen was alway carefully decided before delivery, with a management plan shared between haemostasis experts, obstetrics, and the patients. Considering our experience, the benefit/risk ratio of Hemoleven® should be weighted and management tailored to each individual patient.

Session topic: E-poster

Keyword(s): Bleeding disorder, Factor Xia, Pregnancy

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