PB1531 - Management and outcomes of pregnancy and childbirth in known and unknown haemophilia carriers: a retrospective analysis from a reference Center in Italy.

Background: Guidelines on the optimal management of carriers of hemophilia (HC) for delivery and/or postpartum management are lacking. Data related to the correlation between hemorrhagic symptoms and coagulation FVIII/FIX levels in HC are conflicting. Haemophilia community still debate on the optimal mode of delivery for HC

Aims: This retrospective, observational study aims to describe how known and unkwonn HC were managed during pregnancy and delivery, by evaluating clinical, laboratory parameters and the need for any replacement therapy (RT)

Methods: Following the approval of our Ethics Committee HC were enrolled, through Informed Consent, in a period from January 2022 to October 2022. HC were defined according to the new nomenclature from ISTH.The following data were collected: age, ISTH bleeding score, genetic testing, complete blood cells count, ferritin levels, age of menarche, awareness of HC status, prenatal diagnosis, number and course of pregnancies, type of delivery, fluctuations in FVIII/FIX levels during pregnancy, post-partum bleeding, pregnancy-related complications

Results: Eighteen subjects were enrolled, HC have an increased bleeding risk, regardless of FVIII/FIX levels (Fig.1), instead a significant correlation (p < 0.05) between high body mass index (BMI) and bleeding symptoms is observed (Fig.2). Only two patients were aware of HC status before first pregnancy, however, the others had not been previously evaluated by a specialized center. The most frequent hemorrhages in HCs are secondary to abnormal menstrual blood losses, childbirth, and postpartum. HC enrolled with BMI>30 and FVIII/IX levels above 100% have a higher bleeding tendency than the other HC, they experience moderate-severe hemorrhages requiring treatment. In women with more than one pregnancy, the management of pregnancy and childbirth did not vary after HC status was confirmed

Conclusion(s): HC status is a still inadequately addressed condition, a more accurate report of pregnancy outcomes in HCis needed, a multicenter Italian study on this issue is currently ongoing.