ABSTRACT
BACKGROUND: Hemolytic anemia is rare after liver transplant and is usually associated with ABO mistmatch, post-transplant lymphoproliferative disorders, or medications. CASE REPORTS: We report three patients who had undergone successful liver transplants for primary biliary cirrhosis (PBC) and developed direct antibody test positive autoimmune hemolytic anemia (AIHA) several years into uncomplicated post-transplant management. For two of the patients, the hemolysis responded to steroids and rituximab. One patient required a surgical splenectomy. DISCUSSION: AIHA is an immune-mediated hemolysis that has been reported in patients with PBC. There are no reports of AIHA in patients following liver transplantation for this disease. AIHA should be considered in stable PBC patients who develop anemia years after liver transplant.