Epstein-Barr virus positive post-transplant lymphoproliferative disease (EBV+ PTLD) is a rare, acute, and potentially life-threatening lymphoma that presents challenges in the management of transplant patients.1

There are no EMA- or FDA-approved treatments for EBV+ PTLD. Current treatment approaches include reduction of immunosuppression (RIS), anti-CD20 therapy, and chemotherapy.

EBV+ PTLD after failure of initial treatment can be an aggressive, often deadly disease in which survival can be low, with some patients dying within a few months.2,3

Mechanism of disease of EBV+ PTLD

T-cell suppression plays a significant role in the development of EBV+ PTLD1

Epstein-Barr virus (EBV) is one of the most common human viruses and is associated with more than a dozen malignancies in both hematological and solid tumors.4 EBV is a gamma herpes virus, by which over 90% of the human population is infected.5 Primary infection usually occurs at early age.5

EBV primarily infects B-cells. Primary EBV infection usually occurs prior to adulthood through exposure to saliva or other fluids of an infected individual and results in either asymptomatic infection or symptomatic mononucleosis.5 Following primary infection, EBV establishes a latent infection that persists within memory B-cells at low levels, which results in a lifelong infection that the immune system can usually control but cannot clear.5 In immunocompetent individuals, EBV infection is controlled by a strong immune response of virus-specific and nonspecific T-cells that eliminate the majority of EBV infected B-cells.6

A consequence of EBV infection may include B-cell immortalization.5,7



T-cell suppression plays a significant role in the development of EBV+ PTLD8

Without the T-cells to keep infected cells in check, B-cells may hyperproliferate. In post-transplant patients, immunosuppression may allow for the hyperproliferation of EBV-infected cells, due to reactivation or primary infection, which can lead to EBV+ PTLD.7

Please download a brochure on EBV+ PTLD for more information



Uppdaterad: 28 februari, 2023
1 Dierickx D, Habermann TM. Post-transplantation lymphoproliferative disorders in adults. N Eng J Med 2018;378(6):549-562.
2 Dharnidharka V, Thirumalai D, Jaeger U, et al. Clinical outcomes of solid organ transplant patients with Epstein-Barr virus-driven (EBV +) post-transplant lymphoproliferative disorder (PTLD) who fail rituximab plus chemotherapy: A multinational, retrospective chart review study. Blood 2021;138(Suppl 1):2528.
3 Sanz J, Storek J, Socié G, et al. Clinical outcomes of patients with Epstein-Barr virus-driven post-transplant lymphoproliferative disease following hematopoietic stem cell transplantation who fail rituximab: A multinational, retrospective chart review study. Blood 2021;138 (Suppl 1):1454.
4 Crombie JL, LaCasce AS. Epstein Barr virus associated B-cell lymphomas and iatrogenic lymphoproliferative disorders. Front Oncol 2019;9:109.
5 Nijland ML, Kersten MJ, Pals ST, et al. Epstein-Barr virus-positive posttransplant lymphoproliferative disease after solid organ transplantation: pathogenesis, clinical manifestations, diagnosis, and management. Transplant Direct 2016;2(1):e48.
6 Zimmermann H, Xu H, Barlev A, et al. Clinical outcomes of solid organ transplant patients with EBV+ PTLD who fail first-line rituximab or rituximab plus chemotherapy: an analysis of German PTLD registry. Poster and abstract presented at: European Hematology Association 24th Annual Congress; June 13-16, 2019; Amsterdam, Netherlands.
7 Mrozek-Gorska P, Buschle A, Pich D, et al. Epstein-Barr virus reprograms human B lymphocytes immediately in the prelatent phase of infection. Proc Natl Acad Sci U S A 2019;116(32):16046-16055.
8 Al-Mansour Z, Nelson BP, Evens AM. Post-transplant lymphoproliferative disease (PTLD): risk factors, diagnosis, and current treatment strategies. Curr Hematol Malig Rep 2013;8(3):173-183.
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