Rituximab as therapy for the recurrence of bile salt export pump deficiency after liver transplantation

摘要: Progressive familial intrahepatic cholestasis type 2 (PFIC2) results from recessive mutations in the adenosine triphosphate-binding cassette B11 gene, which encodes for bile salt export pump (BSEP). Liver transplantation (LT) is offered to PFIC2 patients with end-stage liver disease. Reports have described recurrent after transplantation, and this has been associated immunoglobulin G antibodies BSEP. High-titer anti-BSEP appear correlate episodes of cholestatic graft dysfunction. There no established paradigm treating antibody-mediated posttransplant BSEP It appears be refractory changes immunosuppressant medications that would typically effective allograft rejection. Taking what known about its pathophysiology, we designed a treatment consisting rituximab, chimeric monoclonal anti-CD20 antibody, combination intravenous plasmapheresis. Using approach, report successful management recurrence LT.