PB1188 - Pregnancy-associated Acquired Hemophilia A: A Systematic Review

Background: Pregnancy-associated acquired hemophilia A (PAHA) is a rare condition due to the development of autoantibodies against the clotting factor VIII. For this reason, data on PAHA is scarce worldwide.

Aims: We aimed to address clinical, therapeutic, and outcome data to PAHA performing a systematic review.

Methods: The study was conducted following the PRISMA statement. Database-directed search strategies were implemented in March 23. Cohorts and case reports providing individualized data on PAHA were selected by two independent reviewers. Only studies with individualized data were included. (PROSPERO: CRD42022353004)

Results: We selected 4 cohorts (13 patients) and 11 case reports, totaling 24 individual patients. The median age was 31.5 years (interquartile range 28.0-35.0). Three (12%) patients did not receive immunosuppressors and all (100%) reached complete remission (CR) spontaneously. Ten (42%) received immunosuppressors and all (100%) reached CR. No treatment or outcome data were reported for the remaining 11 (46%) patients. Nine newborns were reported for 9 (38%) patients: 3 inhibitor-status unknown without bleeding, 3 inhibitor-status unknown who bled, and 3 inhibitor positive who did not bleed. No deaths were reported for the patients and their children.

Conclusion(s): In this systematic review of individual PAHA cases, CR was reached for those women who were treated with immunosuppressors as well as those who were not. In addition, no death was reported for mothers and children. However, there was a risk of neonatal bleeding.