Emergency ABO-incompatible living donor liver transplantation in Wilson disease-induced acute liver failure
Joseph J Valamparampil1, Deepti Sachan2, Naresh Shanmugam1, Srinivas Mettu Reddy3, Mohamed Rela1
1 Institute of Liver Disease and Transplantation, Bharat Institute of Higher Education and Research, Dr. Rela Institute and Medical Centre, Chennai, Tamil Nadu, India
2 Departments of Transfusion Medicine, Bharat Institute of Higher Education and Research, Dr. Rela Institute and Medical Centre, Chennai, Tamil Nadu, India
3 Department of Liver Transplantation and HPB Surgery, Gleneagles Global Hospital and Health City; Institute of Liver Disease and Transplantation, Chennai, Tamil Nadu, India
Department of Transfusion Medicine, Dr. Rela Institute and Medical Centre, Bharath Institute of Higher Education and Research, No. 07, CLC Works Road, Chromepet, Chennai - 600 044
Source of Support: None, Conflict of Interest: None
We report the clinical outcome of an emergency ABO incompatible-liver transplantation (LT) for an 8-year-old child with Wilson's disease-induced acute liver failure. The pretransplant anti-A antibody titer was 1:64, and hence he underwent three cycles of conventional plasma exchange as pretransplant liver supportive treatment for deranged coagulopathy and liver function followed by one cycle of immunoadsorption (IA) prior to LT. The posttransplant immunosuppression consisted of rituximab, tacrolimus, mycophenolate mofetil, and corticosteroid. The patient had anti-A isoagglutinin rebound with elevated aminotransferases levels from postoperative day 7 for which he was restarted on IA plasmapheresis, but antibody titers did not decrease. Hence, he was switched to conventional plasmapheresis (CP) with which anti-A antibody titers decreased. The total dose of rituximab (150 milligrams/square meter of body surface area) was given in two divided doses of 75 mg at D-1 and D + 8 which was much less than the dose conventionally advocated (375 milligrams/square meter of body surface area). He is clinically well with good graft function without rejection after 1 year of follow-up. This case illustrates that IA and CP in conjunction with adequate immunosuppression is a viable approach in emergency ABO-incompatible-LT in Wilson disease-induced acute liver failure.