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Post-Transplantation Lymphoproliferative Disorders: Diagnosis, Prognosis, and Current Approaches to Therapy

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Abstract

Post-transplantation lymphoproliferative disorders (PTLD) are a heterogenous group of abnormal lymphoid proliferations that occur after solid organ transplant (SOT) or hematopoietic transplantation. PTLDs consist of a disease spectrum ranging from hyperplasia to aggressive lymphomas with 60–70% being Epstein–Barr virus positive. The majority of cases are B-cell, although 10–15% are of T-cell origin or rarely Hodgkin lymphoma. Recent SOT series suggest PTLD occurs at a median of 36–40 months after transplant. Clinically, extra-nodal disease is common (up to 75–85%) including CNS involvement, which is seen in 10–15% of all cases. Since the first report over 40 years ago, PTLD has remained one of the most morbid complications associated with SOT. However, recent data suggests improved survival in the modern era, especially with the integration of early rituximab-based therapy. These studies utilized first line rituximab (+/− chemotherapy) together with reduced immune suppression (RI) for monomorphic and polymorphic PTLD. It will be critical in future studies to determine which PTLDs are most amenable to initial therapy with RI alone, versus RI/rituximab, versus RI/rituximab/chemotherapy. Additionally, novel therapeutics, such as adoptive immunotherapy, should continue to be explored.

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Disclosure

Dr. Evens has been a consultant for and received honoraria from Millennium, Seattle Genetics, Celgene, Pharmacyclics, and Ziopharm.

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Authors and Affiliations

  1. Northwestern University Feinberg School of Medicine, Chicago, IL, USA

    Andrew M. Evens, Rupali Roy, Danielle Sterrenberg, Michelle Z. Moll, Amy Chadburn & Leo I. Gordon

  2. Division of Hematology/Oncology, 676 N. St. Clair Street, Suite 850, Chicago, IL, 60611, USA

    Andrew M. Evens, Rupali Roy & Michelle Z. Moll

  3. Department of Pathology, Chicago, IL, USA

    Amy Chadburn

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Correspondence to Andrew M. Evens.

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Evens, A.M., Roy, R., Sterrenberg, D. et al. Post-Transplantation Lymphoproliferative Disorders: Diagnosis, Prognosis, and Current Approaches to Therapy. Curr Oncol Rep 12, 383–394 (2010). https://doi.org/10.1007/s11912-010-0132-1

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