Abstract Number: PB0725
Meeting: ISTH 2021 Congress
Background: Factor XIII deficiency can present with severe bleeding, intracranial haemorrhages (30%), poor wound healing and spontaneous abortions.
Aims: To present management strategy of a patient with Factor XIII deficiency with Recurrent Miscarriages.
Methods: Hematological, biochemical and imaging studies were done routinely. APLA screen ,Coagulation workup like PT,APTT, factor VIII,VWFAg ,urea clot lysis assay and factor XIII levels were evaluated.
Results: A 27years old woman, had 3rd degree consanguineous marriage had history of 3 consecutive abortions all less than 10 weeks of gestation, came to our institute after 4th spontaneous conception, registered at 7th week of gestation. Clinical Exome sequencing by next generation sequencing, found pathogenic variant of Exon 2 mutation at F13A1 transcript level, homozygous in nature and consistent with Factor XIIIA deficiency.
At 7th week of gestation she gave history of spotting, managed conservatively. Her Factor XIII Ag level was 1.9% and she received 4units of Fresh frozen plasma(FFP). At 14weeks of gestation she was admitted for threatened miscarriage, ultrasound abdomen showed live fetus managed conservatively and discharged. In view of risk of bleeding she was planned for prophylactic FFP transfusion every 2weeks. As she had allergic reactions to FFP, she was switched to cryoprecipitate -5units every 15days. she had regular antenatal followup visits with the obstetrician.
At 36+5 weeks of gestation she delivered a 3.1kg weight female baby by LSCS. During post-partum period, she developed atonic PPH which was managed conservatively. She received 2 days of 5units of cryoprecipitate each day and was discharged on 5th postpartum day in a stable condition.
Conclusions: We successfully used prophylactic FFP & cryoprecipitate in a pregnant patient with factor XIII deficiency and recurrent miscarriages, who had a successful pregnancy outcome with out any complications.
To cite this abstract in AMA style:Surapaneni T, Vadlamani H, Sukayogula M, Ranjith C, Aribandi A. Successful Pregnancy in a Patient with Factor XIII Deficiency and Recurrent Miscarriages [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/successful-pregnancy-in-a-patient-with-factor-xiii-deficiency-and-recurrent-miscarriages/. Accessed November 28, 2023.
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