Posttransplant lymphoproliferative diseases: report from a single center (original) (raw)
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The Journal of heart …, 1991
Patients after solid organ transplantation (SOT) carry a substantially increased risk to develop malignant lymphomas. This is in part due to the immunosuppression required to maintain the function of the organ graft. Depending on the transplanted organ, up to 15% of pediatric transplant recipients acquire posttransplant lymphoproliferative disease (PTLD), and eventually 20% of those succumb to the disease. Early diagnosis of PTLD is often hampered by the unspecific symptoms and the difficult differential diagnosis, which includes atypical infections as well as graft rejection. Treatment of PTLD is limited by the high vulnerability towards antineoplastic chemotherapy in transplanted children. However, new treatment strategies and especially the introduction of the monoclonal anti-CD20 antibody rituximab have dramatically improved outcomes of PTLD. This review discusses risk factors for the development of PTLD in children, summarizes current approaches to therapy, and gives an outlook on developing new treatment modalities like targeted therapy with virus-specific T cells. Finally, monitoring strategies are evaluated.
2010
Post-transplantation lymphoproliferative disorders (PTLDs) are heterogeneous lymphoid proliferations representing a major complication of solid organ transplant. PTLDs are serious complications and may happen at different times after transplantation. Many risk factors are known, such as age, type of organ transplanted, Epstein-Barr virus (EBV) infection before transplant and immunosuppressive therapy. The PTLD incidence in solid organ transplant recipients is variable: from 1% in patients with renal allografts to 6-10% in patients with heart-lung transplantation, and up to 20% in lung transplant recipients. Many reports describe the evolution of PTLDs in heart transplant recipients, but PTLD as an unknown cause of graft dysfunction has never been reported.
Pediatric Transplantation, 2005
Abstract: Post-transplant Lymphoproliferative Disorder (PTLD) because of the Epstein–Barr Virus (EBV) is a major concern after pediatric transplantation. The group at greatest risk is EBV-seronegative recipients who receive EBV-seropositive organs. Additional risk factors remain to be determined, including those among EBV-seropositive recipients. In this case-control study, PTLD cases were biopsy-proven over a period of 4 yr (1997–2000, inclusive). Each case was matched with 2 controls, based on the type of organ transplanted and the period of transplantation (±1 yr). Variables compared between cases and controls included those relating to the clinical and virologic profiles and immunosuppressive therapy. Twenty-two cases of PTLD were diagnosed during the study period. PTLD cases occurred at a median of 22.8 months post-transplantation (range 1–131). The median age of cases was 26.2 months (range 6.1–194) compared with 47.4 months (range 0.8–202.2) for controls (p = 0.93). Cases had a higher mean baseline EBV load compared with controls (3.1 log10 (s.d. ± 1.0) vs. 1.6 log10/106 PBMCs (s.d. ± 1.4), with every 1 log increase in viral load resulting in a three times increase in the likelihood of PTLD (p < 0.007). Close to one in four cases of PTLD were EBV-seropositive pretransplantation. These seropositive recipients tended to be older patients with a trend to a worse outcome compared with their seronegative counterparts. The occurrence of PTLD was not associated with the use of any specific immunosuppressants. A significant proportion of PTLD cases occurred among EBV-seropositive transplant recipients, with a tendency towards an unfavorable outcome. Besides EBV-seronegative recipients who receive seropositive organs, some EBV-seropositive pediatric patients are at risk of PTLD. Additional studies are warranted to further define the factors associated with PTLD in EBV-seropositive transplant recipients.
Transplantation Direct, 2016
Posttransplant lymphoproliferative disease (PTLD) is a potentially fatal complication after (solid organ) transplantation, which is highly associated with Epstein-Barr virus (EBV). The EBV-specific cytotoxic T cell response that is essential in controlling the virus in healthy individuals is suppressed in transplant recipients using immunosuppressive drugs. A primary EBV infection in EBV-seronegative patients receiving an EBV-seropositive donor organ or a reactivation in those who are already latently infected pretransplantation can lead to uninhibited growth of EBV-infected B cells and subsequently to PTLD. Effective preventive strategies, such as vaccines and antiviral agents, are lacking. Because not every transplant recipient with increasing EBV viral load develops PTLD, it is hard to decide how intensively these patients should be monitored and how and when a preemptive intervention should take place. There is a need for other tools to help predict the development of PTLD in patients at risk to make timing and strategy of preemptive intervention easier and more reliable. The cornerstone of the treatment of patients with PTLD is restoring the host's immunity by reduction of immunosuppressive drug therapy. American and British guidelines recommend to add rituximab monotherapy or rituximab in combination with cyclophosphamide, doxorubicin, vincristine, and prednisolone, depending on histology and clinical characteristics. Although response to these therapies is good, toxicity is a problem, and PTLD still has a relatively high mortality rate. An evolving therapy, especially in PTLD occurring in allogeneic stem cell transplantation, is restoring the host's immune response with infusion of EBV-specific cytotoxic T cells. This may also play a role in the future in both prevention and treatment of PTLD in SOT.
Pediatric Transplantation, 2003
Lymphocyte subsets may discern treatment effects in children and young adults with post-transplant lymphoproliferative disorder PTLD is the most frequent tumor developing after organ transplantation. It accounts for about 15-25% of all post-transplant tumors (1). Most PTLD lesions are associated with EBV infection of B-lymphocytes (2). The risk is thought to be the highest in EBV negative recipients who experience primary infection. Although antiviral agents like acyclovir and ganciclovir are used commonly in the treatment of this complication, their efficacy remains unproven (3). Currently, withdrawal or lowering of immunosuppression remains the mainstay of treatment. This often results in acute rejection, which requires that immunosuppression be restarted. In turn, this may lead to exacerbation of the PTLD process, and immunosuppressive therapy may need to be lowered once again. This cycle of events can be repetitive and carries with it a significant potential for patient and graft loss. The prevalence and challenge of managing EBV-associated PTLD have led to a tremendous interest in methods to monitor disease
Pediatric Transplantation, 1999
Abstract: Despite a growing understanding of the pathogenesis and spectrum of Epstein–Barr virus (EBV) and EBV-associated post-transplant lymphoproliferative disease (PTLD) in organ transplant recipients, the optimal management of this complication remains controversial. The absence of comparative data evaluating potential therapeutic strategies explains the lack of uniformly accepted guidelines for the management of PTLD. The purpose of this review is to provide an overview of potential therapies and offer a set of guidelines for the management of EBV-associated PTLD in children.