Epstein-Barr Virus-Associated Post-Transplant Lymphoproliferative Disorders after Hematopoietic Stem Cell Transplantation: Pathogenesis, Risk Factors and Clinical Outcomes

Abstract

Epstein-Barr virus (EBV) is a ubiquitous virus belonging to the human γ-herpes virus subfamily. After primary infection, EBV maintains a life-long latent infection. A major concern is that EBV can cause a diverse range of neoplasms and autoimmune diseases. In addition, patients undergoing hematopoietic stem cell transplantation or solid organ transplantation can experience post-transplant lymphoproliferative disorders (PTLDs) due to dysfunction or suppression of host’s immune system, or uncontrolled proliferation of EBV-infected cells. In recent years, the number of EBV-associated PTLD cases has increased. This review focuses on the current understandings of EBV-associated PTLD pathogenesis, as well as the risk factors and clinical outcomes for patients after allogeneic stem cell transplantation.

Epstein-Barr virus induced post-transplant lymphoproliferative disorder presenting with unilateral retinal involvement

“…PTLD represents a spectrum of lymphoproliferative disorders associated with EBV infection in the setting of pharmacologic immunosuppression 1 , 2 that usually occurs within 1 year of hematopoietic stem cell or solid organ transplantation. 2 Although PTLD is a common condition after hematopoietic stem cell transplantation with a reported incidence ranging from 1 to 17%, 2 , 3 ocular involvement remains a rare finding in this context. 4 …”

Viral Infections in HSCT: Detection, Monitoring, Clinical Management, and Immunologic Implications

“…Immunophenotypically, expression of EBNA 2 and 3 is a characteristic of PTLD ( 91 ). Proliferation of EBNA 3+ lymphoid cells does not occur in the absence of an immunologic impairment, thus linking immunosuppression with PTLD ( 92 ).…”

EBV-Driven Lymphoproliferative Disorders and Lymphomas of the Gastrointestinal Tract: A Spectrum of Entities with a Common Denominator (Part 3)

“…This difference is in part due to the degree of immunosuppressive treatment administered for different SOT. Despite HSCT recipients having a lower risk of PTLD compared to SOT recipients, PTLDs following HSCT show a more aggressive course with high mortality [ 8 , 154 ]. The occurrence of PTLD-like lesions after autologous stem cell transplantation is rare and, according to the current WHO classification, should be included among iatrogenic immunodeficiency-associated LPDs rather than among PTLDs [ 8 ].…”