title: Post-transplant lymphoproliferative disease (PTLD): risk factors, diagnosis, and current treatment strategies.
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).
authors.affiliation: Division of Hematology/Oncology, The University of Massachusetts Medical School, Worcester, 01568, USA.
authors.forename: Zeina
authors.initials: Z
authors.lastname: Al-Mansour
authors.forename: Beverly P
authors.initials: BP
authors.lastname: Nelson
authors.forename: Andrew M
authors.initials: AM
authors.lastname: Evens
mesh_terms: D000368:Aged; D000970:Antineoplastic Agents / Q000627:therapeutic use; D000998:Antiviral Agents / Q000627:therapeutic use; D020031:Epstein-Barr Virus Infections / Q000150:complications; D006801:Humans; D007165:Immunosuppression Therapy / Q000009:adverse effects; D007167:Immunotherapy / Q000379:methods; D008232:Lymphoproliferative Disorders / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D008297:Male; D016377:Organ Transplantation / Q000009:adverse effects*; D012307:Risk Factors; D014184:Transplantation, Homologous / Q000009:adverse effects
chemical_list: D000970:Antineoplastic Agents; D000998:Antiviral Agents