Posttransplant lymphoproliferative disorders (PTLD) comprise a heterogeneous group of diseases spanning a range of pathology and presentation. Epstein Barr virus (EBV) proliferation in B cells and posttransplant immunosuppression critically regulates the pathogenesis of most PTLD. For reasons that are not entirely clear, the incidence of PTLD is relatively high after lung transplant as compared with most other types of solid organ transplant. Recent advances have been made in our understanding of the pathogenesis, diagnosis, and treatment of PTLD in lung transplant recipients and form the basis for this review.
Elucidation of the central role for EBV in PTLD has led to diagnostic strategies focused on detecting the presence of virus through either serologic or polymerase chain reaction–based testing. Although chemotherapy, radiation, and surgery have been historically used in the treatment of PTLD, a dramatic expansion in treatment options with innovative immunotherapies is now occurring. Anti-CD20 antibody, rituximab, has been used in thoracic organ recipients with PTLD and appears quite effective. Adoptive immunotherapy with cytotoxic T lymphocytes (CTL) has limited experience with lung transplant recipients but also appears very promising in the treatment of PTLD. Laboratory studies also suggest rapamycin is useful in patients with PTLD because of its antiproliferative effects on EBV infected B calls as well as its effectiveness in the prevention of rejection, but further confirmation is needed.
As the number of lung transplant recipients grows, so does the potential prevalence of PTLD. The recent introduction of the anti-CD20 monoclonal antibody, rituximab, into clinical practice has already improved our ability to successfully treat PTLD after lung transplant. Ongoing studies of the molecular pathogenesis of B-cell transformation by EBV will likely lead to additional novel immunologically based therapies effective in the prevention or treatment of PTLD.
Department of Medicine, Duke University Medical Center, Durham, North Carolina, USA
Correspondence to Scott M. Palmer, Lung and Heart-Lung Transplant Program, Duke University Medical Center, Box 3876, Durham, NC 27710, USA
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