ObjectiveTo observe the clinical effect of Rituximab combined with intravenous immunoglobulin (IVIG) in preventing blood group antibody mediated rejection (AMR) in pediatric ABO incompatible living donor liver transplantation (ABOi-LDLT).MethodsA total of 503 cases of pediatric living donor liver transplantation in Beijing Friendship Hospital Affiliated to Capital Medical University from June 2013 to December 2020 were retrospectively collected; the overall survival of recipient and graft were compared between ABOi-LDLT and ABO compatible living donor liver transplantation (ABOc-LDLT), and we summarized the data of AMR in 7 cases received Rituximab+IVIG protocol.ResultsThere were 53 cases of ABOi-LDLT and 450 cases of ABOc-LDLT in our study. The 5-year cumulative survival rate of recipients and grafts was 98.0% and 96.0% in the ABOi-LDLT group respectively, and in ABOc-LDLT group was 92.2% and 89.1% respectively, there was no significant difference between the two groups (P=0.232, P=0.381）. Seven children with blood group antibody titer >1∶64 were included in the study. On the basis of classical intensive immunosuppressive therapy, all patients were treated with Rituximab+IVIG. The blood group antibody titer of 6 patients remained stable, and no rejection occurred; one patient developed severe AMR and graft failure, and recovered after salvage treatment of ABOc-LDLT.ConclusionRituximab+IVIG can be used as an effective therapeutic option to prevent blood group AMR after ABOi-LDLT.