Pathology & Oncology Research

, Volume 17, Issue 3, pp 443–454

Lymphoproliferative Disorders After Solid Organ Transplantation—Classification, Incidence, Risk Factors, Early Detection and Treatment Options

Review

DOI: 10.1007/s12253-010-9329-8

Cite this article as:
Végső, G., Hajdu, M. & Sebestyén, A. Pathol. Oncol. Res. (2011) 17: 443. doi:10.1007/s12253-010-9329-8

Abstract

Posttransplant lymphoproliferative disorder (PTLD) is a heterogeneous disease group of benign and malignant entities. The new World Health Organisation classification introduced in 2008 distinguishes early lesions, polymorphic, monomorphic and classical Hodgkin lymphoma-type PTLD. Based on the time of appearance, early and late forms can be identified.

PTLDs are the second most frequent posttransplantation tumors in adulthood, and the most frequent ones in childhood. The incidence varies with the transplanted organ—from 1%–2% following kidney transplantation to as high as 10% following thoracic organ transplantation—due to different intensities in immunosuppression. Immunocompromised state and Epstein-Barr virus (EBV) infection are the two major risk factors.

In Europe and the US approximately 85% of PTLDs are of B-cell origin, and the majority are EBV-associated. Symptoms are often unspecific; extranodal, organ manifestations and central nervous system involvement is common. Early lesions respond well to a decrease in immunosuppression. Malignant entities are treated with rituximab, chemotherapy, radiotherapy and surgical therapy. Adoptive T-cell transfer represents a promising therapeutic approach. The prognosis is favorable in early PTLD, and poor in late PTLD. Five-year survival is 30% for high-grade lymphomas. The prognosis of EBV-negative lymphomas is worse.

Lowering the risk of PTLD may be achieved by low dose maintenance immunosuppression, immunosuppressive drugs inhibiting cell proliferation, and special immunotherapy (e.g. interleukin-2 inhibitors). Early detection is especially important for high risk—e.g. EBV-negative—patients, where the appearance of EBV-DNA and the increase in its titer may help.

Keywords

Adoptive T-cell therapy Early detection Epstein-Barr virus Immunosuppression Lymphoma Posttransplant lymphoproliferative disorders Rituximab Risk factors Solid organ transplantation Therapy 

Abbreviations

ATG

Anti-thymocyte globulin

CMV

Cytomegalovirus

CNI

Calcineurin inhibitor

CNS

Central nervous system

CTL

Cytotoxic T-lymphocyte

DLBCL

Diffuse large B-cell lymphoma

DNA

Deoxyribonucleic acid

EBNA

Epstein-Barr nuclear antigen

EBV

Epstein-Barr virus

FDG-PET

Fluorodeoxyglucose positron emission tomography

HIV

Human immunodeficiency virus

HLA

Human leukocyte antigen

HSCT

Hematopoietic stem cell transplantation

HTLV

Human T-cell leukemia virus

IL

Interleukin

LMP

Latent membrane protein

mTOR

Mammalian target of rapamycin

NK

Natural killer

PCR

Polymerase chain reaction

PSI

Proliferation signal inhibitor

PTLD

Posttransplant lymphoproliferative disorders

SOT

Solid organ transplantation

UNOS

United Network for Organ Sharing

WHO

World Health Organisation

Copyright information

© Arányi Lajos Foundation 2010

Authors and Affiliations

  1. 1.Department of Transplantation and SurgerySemmelweis UniversityBudapestHungary
  2. 2.1st Department of Pathology and Experimental Cancer ResearchSemmelweis UniversityBudapestHungary