Transjugular Intrahepatic Portosystemic Shunt in Liver Transplant Recipients: Single Center Experience With 13 Patients

Abstract

Primary sclerosing cholangitis (PSC) has a high recurrence rate even after liver transplant. High recurrence of PSC after livingrelated liver transplant may be attributed to: (1) homology of HLA (genetic factors of the liver graft) and (2) autoimmune disease. We encountered a male pediatric PSC case without recurrence for 2.5 years after living-related liver transplant and with uneventful followups. This case differed from other PSC cases in two ways: (1) the donor was a fifth-degree relative matching at only one of six HLA loci and (2) rituximab (RTX) was used for ABO-incompatibility. We compared this case with five other PSC cases at our institution and examined preventative measures for recurrences. Subjects: The subjects were 6 PSC patients who had undergone liver transplant. They were 2 males and 4 females. Their ages at transplant ranged from 6 to 42 years. In 5 cases, the donors were parents matching at three of six HLA loci. In the one remaining case, the donor was a female fifth-degree relative matching at only one of six loci. Only this case had AàO incompatibility, and the patient received RTX and intraportal infusion therapy. The other five cases had ABO compatibility. Recurrences were diagnosed by MRCP imaging and liver biopsy. Peripheral blood mononuclear cells of the case with RTX were transplanted into immunodeficient mice. ELISA was used to measure anti-donor antibody production due to sensitization to donor blood-group antigen. Results: In 5 cases with parent donors, recurrences were observed 1-3 years post-transplant. Recurrence was not observed in the rituximab-treated case with a fifth-degree relative donor. The peripheral B-cells of this case did not produce anti-donor blood group antibodies due to sensitization. Discussion: The following were indicated to be possible preventative measures against PSC recurrence: (1) selection of a donor who is unrelated, preferably with HLA mismatched and (2) the use of rituximab at liver transplant to induce B-cell tolerance and to prevent autoimmune disease recurrences.Disclosure: All authors have declared no conflicts of interest.