Background: Patients with congenital hypofibrinogenemia/afibrinogenemia (CH/CA) maybe with various bleeding symptoms proportional to the low values of functional and antigenic fibrinogen (FIB) or may have thrombotic complications. FIB is important in maintaining placenta integrity by supporting cytotrophoblast.

Aims: We present a case of congenital afibrinogenemia with two favored pregnancy outcomes.

Methods: .

Results: Case: A 31-year-old woman with excessive uterine bleeding and recurrent vulvar hematomas had prolonged aPTT/PT (APTT:>160sec, PT:108sec) and low FIB (<25mg/dl) with normal d-dimer level. CA was thought of as a diagnosis and cryoprecipitate were given as an emergent replacement. Further evaluation could not be made: The patient was lost of follow-up. She admitted 6 years later as being in the 5th gestational week. She described 5 early pregnancy losses and had a highly consanguineous family(Fig.1). One of her 6 siblings had also CA. Regular replacement therapy to keep the FIB level >75mg/dL with FIB concentrates or cryoprecipitate infusions throughout pregnancy was started. On the 36th week, with a FIB level of 150mg/dl, a spontaneous vaginal delivery successfully occurred. The infant appeared well, had normal vital signs, and good perfusion. Bodyweight was 2950g with an Apgar score of 9/10. Fibrinogen replacement was discontinued. Two years later, she was pregnant again on the 5th week. She commenced regular replacement therapy for increasing FIB. The second pregnancy was also ended favorably by spontaneous vaginal delivery. The infant’s body weight was 3210g. No postpartum complications were experienced. The patient continued to breastfeed for one year period after her first delivery. She continues breastfeeding after her 2nd delivery without any replacements.

Pedigree of the patient

Conclusions: CH/CA or dysfibrinogenemia may result in obstetric complications such as first-trimester pregnancy loss, hemorrhage, placental abruption, and thrombosis. Our main strategy was FIB replacement from the beginning of the pregnancy until delivery with a target FIB level of 60 – 100mg/dL to manage CH/CA patients.

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ISTH Congress Abstracts - https://abstracts.isth.org/abstract/a-case-of-congenital-afibrinogenemia-with-two-successful-spontaneous-delivery-after-recurrent-pregnancy-losses/