Early-onset plasma cell-rich acute cellular rejection following liver transplantation in autoimmune hepatitis-primary biliary cirrhosis overlap syndrome: a case report

Plasma cell-rich rejection (PCRR) is a distinct form of liver allograft rejection marked by dense plasma cell infiltration and features resembling autoimmune hepatitis. It typically presents months after transplantation and is associated with poor graft outcomes. A 36-year-old female with autoimmune hepatitis-primary biliary cirrhosis overlap underwent deceased donor liver transplantation with an uneventful early postoperative course. On postoperative day (POD) 27, she developed high-grade fever and marked transaminitis. Liver biopsy at POD 27 revealed acute cellular rejection (rejection activity index 3/9), while a repeat biopsy at POD 131 revealed prominent plasma cell infiltration suggestive of PCRR in the setting of subtherapeutic tacrolimus levels. Immunosuppression was optimized, leading to transient improvement. On POD 161, she developed seizures and altered sensorium; evaluation showed cryptococcal meningitis and cytomegalovirus infection. Repeat biopsy demonstrated evolving chronic rejection with ductopenia and centrilobular cholestasis. Despite intensive antimicrobial therapy and immunosuppressive adjustments, her condition worsened, resulting in death on POD 204. Although PCRR usually presents late, early atypical presentations may occur. This case highlights the importance of recognizing PCRR on biopsy, along with early histological evaluation, strict immunosuppressive monitoring, and timely intervention to improve outcomes.