Primary sclerosing cholangitis and other risk factors for post-transplant lymphoproliferative disease after liver transplantation in adults.

Post-transplant lymphoproliferative disease (PTLD) is a rare but serious complication of liver transplantation (LT) with morbidity and mortality. The risk factors for PTLD in adults are ill-defined. The current study aimed to assess the risk factors for PTLD after LT in adults. All adult LT recipients between 1986 and 2016 from two centers in the Netherlands were included, with follow-up until 2020. PTLD was diagnosed according to the WHO classification. Potential risk factors for PTLD were assessed using multivariate Cox regression analysis. 1281 patients were included, of whom 29 (2.3%) developed PTLD. Results show that independent risk factors for PTLD after LT in adults were no Epstein-Barr virus (EBV) load monitoring strategy, primary sclerosing cholangitis (PSC) as an indication for LT, era (historic era linked to more intense long-term immunosuppression), and EBV seronegative recipient. No other independent risk factors were identified in this study. Of 207 patients with PSC as an indication for LT, 13 (6.3%) developed PTLD versus 16 out of 1074 (1.5%) patients with other underlying liver diseases (log-rank p<0.001). The yearly PTLD incidence was higher in the first year than in the later years after LT (2.4%/year vs. 0.6%/year) for PSC but not for other indications (0.16%/year). In EBV seronegative recipients, PTLD occurred earlier after LT, while in the 97% seropositive recipients it could occur very late after LT.