Introduction: Antibody mediated rejection (AMR) is fewer than T cell mediated rejection in pediatric liver transplantation. However, it is difficult to make the precise and timely diagnose of AMR, until combination of clinical manifestations, histopathology, C4d staining and donor specific antibody (DSA). Our aim is to summary the diagnosis and prognosis of AMR in pediatric recipients after liver transplantation (LT), especially one unique type of AMR manifesting refractory ascites.
Methods: Total 15 pediatric recipients received LT with biliary atresia were included in our study. When graft dysfunction occured, repeated liver biopsies were performed and donor specific antibody (DSA) was detected by Luminex.
Results: Thirteen of the fifteen cases appeared abnormal graft function at 1.6 to 79.4 months post-LT, compared by two cases with hypoproteinemia and refractory ascites at 38 months and 50 months post-LT. Furtherly, ultrasound, enhanced computed tomography and hepatic venous angiography showed normal blood flow in two recipients with ascites. DSA in all patients were strongly positive (MFI > 10,000) and against at HLA-II antigens (4 cases at DR and 13 cases at DQ). Staining of C4d were positive in 10 cases by repeated liver biopsies, but negative in 5 cases. Combined with histopathology, AMR was confirmed and the patients were received mycophenolate mofetil, rituximab and intravenous immunoglobulin. More than half of the recipients diagnosed AMR were cured well by conservative treatments and one patient with refractory ascites was sustained. However, one third of the patients’ conditions haven’t improved after treatments, through MFI of DSA decreased. Toatal 5 patients were received re-transplantation and showed a well graft function for more than 3 years in follow-up.
Conclusion: AMR including which was characterized by ascites was rare but the prognosis was poor in pediatric LDLT, even with conventional therapies. Re-transplantation is one of the valid treatment strategies, in case of patient’s condition can’t be maintained by conservative therapies.