Post-transplant lymphoproliferative disease (PTLD): risk factors, diagnosis, and current treatment strategies

Curr Hematol Malig Rep. 2013 Sep;8(3):173-83. doi: 10.1007/s11899-013-0162-5.

Abstract

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).

Publication types

  • Review

MeSH terms

  • Aged
  • Antineoplastic Agents / therapeutic use
  • Antiviral Agents / therapeutic use
  • Epstein-Barr Virus Infections / complications
  • Humans
  • Immunosuppression / adverse effects
  • Immunotherapy / methods
  • Lymphoproliferative Disorders* / diagnosis
  • Lymphoproliferative Disorders* / etiology
  • Lymphoproliferative Disorders* / therapy
  • Male
  • Organ Transplantation / adverse effects*
  • Risk Factors
  • Transplantation, Homologous / adverse effects

Substances

  • Antineoplastic Agents
  • Antiviral Agents