Pregnancy outcomes among women with inherited bleeding disorders: A matched case-control study.

BACKGROUND: Inherited bleeding disorders (IBDs) are uncommon but clinically important conditions in pregnancy because they may increase maternal bleeding risk and may also affect fetal and neonatal outcomes. This study evaluated maternal, fetal, and neonatal outcomes among pregnant women with IBDs at a tertiary referral center in Iran. METHODS: This retrospective matched case-control study was conducted between January 2010 and December 2020. Eighty-one pregnancies among women with hematology-confirmed IBDs were matched to 162 control pregnancies without documented bleeding disorders. The primary outcome was fetal/neonatal mortality, defined as stillbirth/intrauterine fetal death or neonatal death. Secondary outcomes included NICU admission, IUGR, neonatal blood transfusion, postpartum bleeding, postpartum hemorrhage, delivery mode, and hemostatic interventions. RESULTS: The most common diagnostic category identified was other inherited factor deficiencies, followed by von Willebrand disease, hemophilia A-related disorders/carrier status, Glanzmann thrombasthenia, and hemophilia B-related disorders/carrier status. The rates of stillbirth/IUFD were significantly higher in pregnancies among women with IBDs at 11.1% compared to 0.6% in controls (relative risk [RR] 18.00, 95% confidence interval [CI] 2.32-139.65). Similarly, neonatal death occurred more frequently in women with IBDs (7.4% vs. 0.6%; RR 12.00, 95% CI 1.47-98.01). The composite rate of fetal and neonatal mortality was also elevated among pregnancies affected by IBDs compared to controls (18.5% vs. 1.2%; RR 15.00, 95% CI 3.51-64.02). However, rates of NICU admission and IUGR were not significantly increased among these cases. Additionally, among women with IBDs, postpartum bleeding was reported in 25.9%, postpartum hemorrhage in 14.8%, and at least one hemostatic intervention was documented in 77.8% of cases. CONCLUSION: Pregnancies among women with IBDs were associated with increased fetal/neonatal mortality and substantial maternal bleeding-related morbidity in this tertiary-center cohort. These findings support early diagnosis, multidisciplinary antenatal planning, individualized delivery management, access to hemostatic therapy, and structured neonatal assessment for pregnancies affected by IBDs.