Classification and Treatment of Posttransplant Lymphoproliferative Disorders

Abstract

Posttransplant lymphoproliferative disorders (PTLDs) are defined by the 2008 World Health Organization classification. Monomorphic PTLD is the most frequent form; it is usually diagnosed several years after transplantation and nowadays is positive for Epstein–Barr virus in only 50% of cases. Although preventive treatments are not effective, in cases of Epstein–Barr virus reactivation, a preemptive approach can prevent the development of PTLD. The first-line curative treatment consists of reducing immunosuppression, where possible, and this alone can cure PTLD. If this fails, rituximab monotherapy is safe and induces complete remission in one-third of cases. If complete remission is not achieved, four cycles of R-CHOP [cyclophosphamide, hydroxy doxorubicin, vincristine (Oncovin®), and prednisone plus rituximab] are generally sufficient.