Current Hematologic Malignancy Reports

, Volume 8, Issue 3, pp 173–183 | Cite as

Post-Transplant Lymphoproliferative Disease (PTLD): Risk Factors, Diagnosis, and Current Treatment Strategies

  • Zeina Al-Mansour
  • Beverly P. Nelson
  • Andrew M. EvensEmail author
Lymphomas (C Dearden, Section Editor)


Post-transplant lymphoproliferative diseases (PTLD) are heterogeneous lymphoid disorders ranging from indolent polyclonal proliferations to aggressive lymphomas that complicate solid organ or hematopoietic transplantation. Risk factors for PTLD include viral infections, degree of immunosuppression, recipient age and race, allograft type, and host genetic variations. Clinically, extra-nodal disease is common including 10–15 % presenting with central nervous system (CNS) disease. Most PTLD cases are B cell (5-10 % T/NK cell or Hodgkin lymphoma), while over one-third are EBV-negative. World Health Organization (WHO) diagnostic categories are: early lesions, polymorphic, and monomorphic PTLD; although in practice, a clear separation is not always possible. Therapeutically, reduction in immunosuppression remains a mainstay, and recent data has documented the importance of rituximab +/- combination chemotherapy. Therapy for primary CNS PTLD should be managed according to immunocompetent CNS paradigms. Finally, novel treatment strategies for PTLD have emerged, including adoptive immunotherapy and rational targeted therapeutics (e.g., anti-CD30 based therapy and downstream signaling pathways of latent membrane protein-2A).


Post-transplant lymphoproliferative disease PTLD Lymphoma Non-Hodgkin lymphoma Hodgkin lymphoma Central nervous system CNS Lymphoma Epidemiology Risk factors Diagnosis Pathogenesis Genetic variation EBV LMP2A Prognosis Treatment Rituximab Adoptive immunotherapy Chemotherapy 


Conflict of Interest

Z. Al-Mansour declares that she has no conflict of interest.

B. Nelson declares that she has no conflict of interest.

A. Evens: Consultant for Seattle Genetics and Millennium.

This article does not contain any studies with human or animal subjects performed by any of the authors.


Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  • Zeina Al-Mansour
    • 1
  • Beverly P. Nelson
    • 2
  • Andrew M. Evens
    • 1
    Email author
  1. 1.Division of Hematology/OncologyThe University of Massachusetts Medical SchoolWorcesterUSA
  2. 2.Department of PathologyNorthwestern University Feinberg School of MedicineChicagoUSA

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