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A Patient with Congenital Hypofibrinogenemia who Showed Embryo Implantation, Continuation of Pregnancy, and Delivery Following Continuous Transfusion of Dry Human Fibrinogen Preparations

Y. Seki1, T. Ushiki2, M. Masuko3, J. Takizawa3, H. Sone3, N. Okumura4

1Niigata University/Department of Hematology, Uonuma Institute of Community Medicine, Minami-Uonuma, Japan, 2Niigata University/Blood transfusion, Niigata, Japan, 3Niigata University/Department of Hematology, Niigata, Japan, 4Shinsyu University/Department of Health Science, Matsumoto, Japan

Abstract Number: PB0402

Meeting: ISTH 2021 Congress

Theme: Fibrinogen, Fibrinolysis and Proteolysis » Fibrinogen and Factor XIII

Background: We report a patient with congenital hypofibrinogenemia showing embryo implantation, continuation of pregnancy, and delivery following the continuous transfusion of dry human fibrinogen preparations.

Aims: [Case report] A 38-year old woman underwent embryo transfer (pre-pregnancy fibrinogen [Fbg] activity and antigen levels: 33 and 91 mg/dL, respectively). During the 2nd embryo transfer (failure), the Fbg level just after Fbg supplementation was 145 mg/dL and 66 mg/dL on day 5. During the third attempt (successful), we supplemented 4 g of Fbg 2 days before the embryo transfer (day 0) and at days 2, 7, and 12 for stabilizing the endometrium. The Fbg levels at implantation were maintained at 150 mg/dL. In the early phase of pregnancy, we supplemented 4 g of Fbg (thrice/2 weeks) regularly; Fbg levels were maintained at > 150mg/dL. From the 32nd week of pregnancy, the baby gained weight and the Fbg levels decreased. Then, we supplemented 3 g of Fbg (thrice/week); Fbg levels were maintained at 130-150 mg/dL. Because the Fbg levels decreased from the 34th week, we supplemented 13 g of Fbg/week. A child was born in the 35th week via cesarean operation.

Methods: [Gene analysis] Genetic anlysis of the DNA base sequences of the PCR products of the Aα, Bβ, and γ chains confirmed heterozygosity, with the nucleotides TGT (normal, wild-type) and CGT (morph), which encoded the 326thamino acid in the γ chain of exon 8. Further, cysteine (normal, wild-type) was replaced with arginine (morph); thus far, no studies have reported this finding.

Results: [Discussion] Although Fbg is not reported to be necessary during implantation in natural pregnancies, herein, implantation was successful in the presence of Fbg levels > 150 mg/dL during embryo transfer.

Conclusions: Fbg levels > 150 mg/dL may be necessary for implantation during embryo transfer in patients with congenital hypofibrinogenemia.

To cite this abstract in AMA style:

Seki Y, Ushiki T, Masuko M, Takizawa J, Sone H, Okumura N. A Patient with Congenital Hypofibrinogenemia who Showed Embryo Implantation, Continuation of Pregnancy, and Delivery Following Continuous Transfusion of Dry Human Fibrinogen Preparations [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/a-patient-with-congenital-hypofibrinogenemia-who-showed-embryo-implantation-continuation-of-pregnancy-and-delivery-following-continuous-transfusion-of-dry-human-fibrinogen-preparations/. Accessed September 27, 2023.

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ISTH Congress Abstracts - https://abstracts.isth.org/abstract/a-patient-with-congenital-hypofibrinogenemia-who-showed-embryo-implantation-continuation-of-pregnancy-and-delivery-following-continuous-transfusion-of-dry-human-fibrinogen-preparations/

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