Lymphoproliferative disorders after solid organ transplantation- classification, incidence, risk factors, early detection and treatment options

Gyula Végso, Melinda Hajdu, A. Sebestyén

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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.

Original languageEnglish
Pages (from-to)443-454
Number of pages12
JournalPathology and Oncology Research
Volume17
Issue number3
DOIs
Publication statusPublished - Sep 2011

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Lymphoproliferative Disorders
Organ Transplantation
Human Herpesvirus 4
Immunosuppression
Incidence
Therapeutics
Epstein-Barr Virus Infections
Adoptive Transfer
Immunosuppressive Agents
Hodgkin Disease
Kidney Transplantation
Non-Hodgkin's Lymphoma
Immunotherapy
Lymphoma
B-Lymphocytes
Radiotherapy
Thorax
Central Nervous System
Maintenance
Cell Proliferation

Keywords

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

ASJC Scopus subject areas

  • Cancer Research
  • Oncology
  • Pathology and Forensic Medicine

Cite this

@article{135a0b7c3d5a45b1b163d3c35b109bec,
title = "Lymphoproliferative disorders after solid organ transplantation- classification, incidence, risk factors, early detection and treatment options",
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.",
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AU - Hajdu, Melinda

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