Objective: Pregnancies at risk of early-onset severe hemolytic disease of the fetus and newborn (EOS-HDFN) are at high risk of poor outcomes. Standard-of-care (SOC) for pregnancies at risk of EOS-HDFN includes non-invasive monitoring for fetal anemia and intrauterine blood transfusions (IUTs) if fetal anemia manifests. Intravenous immunoglobulin (IVIG) or plasmapheresis may be administered to delay onset of fetal anemia and the need for IUT. The CLARITY trial aims to characterize the current SOC, clinical course, and outcomes at global HDFN referral centers for pregnant individuals and their offspring at high risk for EOS-HDFN.
Methods: CLARITY (NCT03755128), a prospective, global, multicenter, observational study, enrolled 15 alloimmunized pregnant participants with singleton pregnancies at high risk for EOS-HDFN based on a prior obstetric history of fetal loss, fetal hydrops, or severe fetal anemia at ≤24 weeks gestational age (GA) and a titer in the current pregnancy of >32 anti-D or >4 anti-Kell with confirmation of an antigen. The primary outcome is the proportion of participants with a live birth at ≥32 weeks GA without an IUT. Other perinatal and neonatal outcome variables are evaluated up to Day 28 postpartum of the last maternal participant.
Results: Primary outcome was achieved by 2/15 (13%) pregnant participants enrolled. 14/15 (93%) pregnancies resulted in a live birth, with a median GA at delivery of 36 2/7 weeks (31 4/7 to 38 0/7). Overall, 13/15 (87%) participants required IUTs, with a median of 4 (1-11) IUTs. The median GA at first IUT was 24 0/7 weeks (13 0/7 to 28 2/7). There was 1 (7%) case of hydrops fetalis. One pregnancy resulted in fetal demise which occurred at 16 5/7 weeks. Postnatal simple transfusions were required by 8/14 (57%) neonates/infants and exchange transfusions were required by 3/14 (21%). 4/13 (31%) maternal participants treated with IUT experienced complications. 7/15 (47%) maternal participants received IVIG. The 2 pregnant participants achieving primary outcome received IVIG. 3/7 (43%) participants who received IVIG experienced IVIG-related complications.
Conclusion: This prospective, observational study demonstrates that pregnancies at high risk for EOS-HDFN managed by SOC continue to experience significant unmet needs. Almost all required multiple IUTs, often starting <24 weeks GA, with a high complication rate even if IVIG was administered. These results highlight the medical need for an effective, non-invasive intervention for the treatment and management of pregnant individuals and their offspring at high risk for EOS-HDFN.