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.
Highlights
Post-transplant lymphoproliferative disease (PTLD) is a significant complication of solid organ transplantation (SOT), and it is the most common post-transplant malignancyChildren 2021, 8, 661 in children
It was considered useful to design a survey whose results could reveal the degree of agreement between various pediatric SOT programs on aspects related to the prevention, diagnosis, and treatment of PTLD
We can consider the response to treatment recorded in the cases included in this series as very good, with a survival rate of 88% of the patients (160/181), it should be noted that one-third developed graft rejection as a consequence of the reduction or cessation of the IS. These results indicate that the general prognosis of PTLD in children with SOT is globally good; there is still room for improvement in terms of patient survival as well as in relation to potential complications derived from treatment
Summary
Post-transplant lymphoproliferative disease (PTLD) is a significant complication of solid organ transplantation (SOT), and it is the most common post-transplant malignancyChildren 2021, 8, 661 in children. Post-transplant lymphoproliferative disease (PTLD) is a significant complication of solid organ transplantation (SOT), and it is the most common post-transplant malignancy. PTLD is largely a disease of the modern medical era, directly linked to the use of increasingly potent immunosuppressive regimens [1,2]. The risk factor profile and pathogenesis of PTLD are not completely defined. Is associated with PTLD, not all patients with high viral load develop this malignancy, and some PTLD tumors are EBV-negative. These apparent contradictions make it difficult to predict which patients will develop PTLD, of what severity or site and how they will respond to treatment [3,4]. Risk factors for SOT–PTLD include EBV recipient–donor seromismatch
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