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.