Increased Incidence of Post-transplant Lymphoproliferative Disorder in Autoimmune Liver Disease: An Irish National Experience

Abstract

Post-Transplant Lymphoproliferative Disorder (PTLD) is a well-recognized complication post solid organs transplant. PTLD represents a broad spectrum of abnormalities ranging from an infectious mononucleosis like illness to malignant lymphoma. A retrospective study was performed by collecting data of orthotopic liver transplant (OLT) patients in the National Liver Unit in Ireland from December 1993 to December 2014. Data was analyzed to identify PTLD patients and determine their demographic details, the indication for liver transplant, presenting symptoms, immunosuppression regimens, Epstein"Barr virus (EBV) status and PTLD outcome. From a total of 756 liver transplants recipients, 20 patients (2.6%) were diagnosed with PTLD. The median time from OLT to PTLD diagnosis was 83 months. The main primary indication for OLT of the PTLD cohort was Autoimmune Liver Disease (AiLD) (n=13, 65%, mainly primary sclerosing cholangitis (PSC) n=8, 40%). The combined group of auto-immune hepatitis, PSC and primary biliary cholangitis had a significantly higher incidence of PTLD compared to other etiologies (P<0.01). In AiLD PTLD subgroup, 61.5% were positive for EBV. Five patients (38.5%) had extra-nodal disease and 3 patients had CNS disease. 61% of PTLD AiLD patients (n=8) achieved complete response following their treatment. PTLD has high mortality however early diagnosis and complete remission are achievable. Our study suggests that the incidence of PTLD is increased in AiLD and notably PSC patients.