Posttransplant lymphoproliferative disorder in adult liver transplant recipients: a report of seventeen cases.

Posttransplant lymphoproliferative disorder (PTLD) is a major complication of liver transplantation, but previous descriptions have been limited to case reports and small case series. We report a retrospective analysis of 17 consecutive cases of PTLD associated with liver transplantation. The median age at PTLD diagnosis was 47 years (range 19 - 63) with a median time of 25 months from liver transplantation to PTLD diagnosis (range 3 - 75). PTLD location was frequently extranodal (71%) and involved the transplanted liver (41%). PTLD histology consisted of nine (53%) monomorphic and eight (47%) polymorphic disease. EBV was present by in situ hybridization in 11 (79%) of 14 cases evaluated. Initial therapy included reduction in immunosuppression (RI) alone in 13 (76%) of 17 patients, resulting in 6 (46%) complete responses (CR) and 7 (54%) progressive disease (PD). Monoclonal CD20 antibody (rituximab) and CHOP chemotherapy were used as initial therapy or as second line after RI failure. Currently, five patients (29%) are alive in CR. Although detection and treatment of PTLD in liver transplant recipients remains problematic and upfront mortality is still high, long-term survival is possible. Further studies are necessary to better define treatment strategies.