PB1189 - Treatments and outcomes of acquired hemophilia A among women during the postpartum period: a systematic review

Background: Acquired hemophilia A (AHA) is a rare bleeding disorder due to the development of neutralizing autoantibodies against factor VIII (FVIII), called inhibitors. AHA may be associated to postpartum period. However, data about postpartum-associated AHA are scarce.

Aims: We reported the preliminary results of a systematic review concerning the treatment and outcomes of postpartum-associated AHA.

Methods: The study was conducted following PRISMA statement. Database-directed search strategies were performed in March/23. Publications reporting women during the postpartum period with AHA were included. Only individual data were extracted. We did not include group-reported data. (PROSPERO CRD42022353004)

Results: Forty-three publications described 89 individual postpartum-associated AHA. Median age was 29.0 years (range 18.0-44.0; n = 90). Among the 88/89 (98%) patients who bled, 34 /89 (38%) received red blood cell transfusion and 36/89 (40%) received bypassing agents. Immunosuppression was prescribed to 80/89 (89%). Complete remission (CR) was achieved in 67/89 (75%) patients: spontaneously in 8/89 (8%) patients and after immunosuppression in 59/89 (66%). For patients who achieved CR spontaneously, the median interval between delivery and CR was 14 months (range 7.0-27.0; n = 5). Among the immunosuppression-treated patients, the median interval between delivery and CR was 12.1 months (IQR 2.0-65.9; n = 8), the median interval between diagnosis and CR was 6.6 months (IQR 0.6-131.0; n = 24), and the median between the start of IS treatment and CR was 1.8 month (IQR 0.4-16.0; n = 7). There were 5/90 (6%) deaths (4 bleed-related). Five neonates were described as inhibitor-positive.

Conclusion(s): Immunosuppression in postpartum-associated AHA was associated with a high prevalence of CR, CR could be reached spontaneously. Nevertheless, the risk of death was high.