An 18-year-old woman two years post-heart transplant presents with fever, weight loss and adenopathy. You suspect that she has post-transplant lymphoproliferative disease (PTLD).
What are the risk factors for PTLD? What therapeutic interventions are available?
Post-transplant lymphoproliferative disease (PTLD) refers to a heterogeneous group of lymphoproliferative diseases. These are often, but not always, associated with the Epstein-Barr virus (EBV). Patients who are serologically negative for EBV and receive an EBV-positive organ (EBV mismatch) are at an increased risk of PTLD; for this reason, pediatric patients are a higher-risk subgroup. Cytomegalovirus and hepatitis C may also be risk factors. Use of lymphocyte-depleting antibody therapy and the total degree of immunosuppression are also risk factors.
There are a variety of histologic lesions in PTLD, including an infectious mononucleosis-like picture, polymorphic PTLD, Hodgkin’s lymphoma-like PTLD and monomorphic PTLD that can appear like a B-cell, T-cell or natural killer lymphoma.
Treatment of PTLD depends on the severity of the histologic lesion and the presence or absence of EBV in the abnormal cells. Reduction of immunosuppression alone may result in improvement of early stage lesions. For EBV-positive cases, ganciclovir or valganciclovir have been used. For true lymphomas, chemotherapy is usually indicated, often combined with rituximab in the case of B-cell lymphomas.