Current Practices on Diagnosis, Prevention and Treatment of Post-Transplant Lymphoproliferative Disorder in Pediatric Patients after Solid Organ Transplantation: Results of ERN TransplantChild Healthcare Working Group Survey

Abstract

(1) Background: Post-transplant lymphoproliferative disease (PTLD) is a significant complication of solid organ transplantation (SOT). However, there is lack of consensus in PTLD management. Our aim was to establish a present benchmark for comparison between international centers and between various organ transplant systems and modalities; (2) Methods: A cross-sectional questionnaire of relevant PTLD practices in pediatric transplantation was sent to multidisciplinary teams from 17 European center members of ERN TransplantChild to evaluate the centers’ approach strategies for diagnosis and treatment and how current practices impact a cross-sectional series of PTLD cases; (3) Results: A total of 34 SOT programs from 13 European centers participated. The decision to start preemptive treatment and its guidance was based on both EBV viremia monitoring plus additional laboratory methods and clinical assessment (61%). Among treatment modalities the most common initial practice at diagnosis was to reduce the immunosuppression (61%). A total of 126 PTLD cases were reported during the period 2012–2016. According to their histopathological classification, monomorphic lesions were the most frequent (46%). Graft rejection after PTLD remission was 33%. Of the total cases diagnosed with PTLD, 88% survived; (4) Conclusions: There is still no consensus on prevention and treatment of PTLD, which implies the need to generate evidence. This might successively allow the development of clinical guidelines.

Late airway complications following pediatric liver transplantation: A case series

“…5,24 Risk factors for the development of PTLD include EBV-naïve recipient less than 12 months of age, use of anti-thymocyte globulin, high levels of immunosuppression and early episodes of acute rejection. 25,26 Notably, transplantation at less than 12 months of age is a risk factor for both eosinophilic gastrointestinal disease and PTLD. 11 Additionally, tacrolimus, as a more potent agent compared to the older calcineurin inhibitor agent ciclosporin, was found to be an independent risk factor in the precipitation of PTLD in both pediatric liver and heart transplant recipients.…”

Post-transplant lymphoproliferative disease after pediatric kidney transplant

“…This was highlighted in one study of 276 pediatric kidney transplant recipients in which EBV-negative recipients of EBV-positive donor organs demonstrated a 6-fold higher risk of PTLD ( 15 ). The highest incidence of PTLD occurs at primary EBV seroconversion due to de novo infection or when it is acquired from passenger lymphocytes in the graft ( 16 ). Both primary EBV infections in EBV-negative patients and reactivation in EBV-positive individuals with latent infection can lead to uninhibited growth of EBV-infected B-cells and the development of PTLD ( 17 ).…”

“…Reduction of immunosuppression (RIS) is the cornerstone of management for all types of PTLD, with the goal of restoring EBV-specific cellular immunity without increasing the risk of acute rejection. This remains the most common practice at diagnosis of PTLD in pediatric SOT recipients ( 16 , 24 ). Generally, immunosuppression is reduced to the lowest tolerable level, and at times can reach as low as 25%–50% of baseline therapy.…”

Recent Advances in Adult Post-Transplant Lymphoproliferative Disorder

“…In this context, a variety of therapeutic options have been proposed including chemo- and immunotherapy, radiation therapy, and various novel treatments, all with variable results [ 7 ]. While there are no guidelines for treatment, the type of lesion and EBV status generally drives the type of therapy based on consensus statements [ 8 , 9 , 10 ]. Further research is necessary to establish concrete treatment recommendations.…”