Joining Efforts for PTLD: Lessons Learned from Comparing the Approach and Treatment Strategies Across the Pediatric and Adult Age Spectra

Montanari, Francesca; Orjuela‐Grimm, Manuela

doi:10.1007/s11899-021-00606-8

Cited by 8 publications

(22 citation statements)

References 38 publications

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“…Due to the heterogenic nature of PTLD, the therapeutic approach needs to consider the histology and clinical settings in which PTLD has developed [34]. Reduced immunosuppression (RI) or discontinuation of the IS is recommended earlier for all cases, and this was a universal practice in the centers included in our study.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2021

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(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.

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Section: Discussionmentioning

confidence: 99%

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

et al. 2021

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“…The success rate in monotherapy in adult patients was variable, with 42% of patients achieving complete response, 17% partial response, and with progressive disease in 41% of patients. The achieved remissions were relatively short-term, and 26% of responders were re-treated within 1 year; however, this improved the rates of complete response in 61% of partial responders [3]. Rituximab in monotherapy induced a significant decrease of EBV load to an undetectable level in EBVpositive pediatric patients after kidney transplantation; however, this effect was time-limited and disappeared within 2-6 months, and viral load returned to baseline in most of the cases.…”

Section: Treatmentmentioning

confidence: 87%

“…The data vary between different databases, settings, and transplantation eras, and the relevant numbers are limited to the whole group of PTLD. The overall 10-year incidence of PTLD in adult SOT recipients reported by UNOS (United Network for Organ Sharing) was 0.7% in kidney, 1% in liver, 1% in heart and pancreas, and 2% in lung transplantation [3]. The highest incidence of PTLD (19%) was reported in intestinal and multiorgan transplantation, which could be translated to almost 20 times higher relative risk compared with kidney transplantation (239.5 vs. 12.6) [4].…”

Section: Incidencementioning

confidence: 99%

“…The usual factors of poor prognosis identified in adult patients, including stage III/IV of malignancy, involvement of the allograft, and elevated LDH, should predict a lack of response to RI [51,52]. In children, poor predictors of response to RI included EBV and B CD20 negativity, lateonset PTLD, CNS involvement, and Burkitt or Hodgkin lymphoma morphology [2,3]. The next step includes B CD20 cell depletion by anti-CD20 antibody.…”

Section: Treatmentmentioning

confidence: 99%

“…There were published data on its efficacy in kidney and liver transplant recipients in terms of primary infection and increased clearance of EBV [61][62][63]; however, a recent pediatric report denied any effect of 3-month valganciclovir prophylaxis (otherwise applied as anti-CMV prophylaxis) on the risk of PTLD [27]. Tumor cells latently infected by EBV do not express specific viral protein kinase, crucial for drug activity [2,3]. It was the basis of a preliminary (adult) study, involving the induction of viral lytic cycle with arginine butyrate (as the inductor) and using ganciclovir in this condition in adult patients (including SOT, 1 kidney) with different types of lymphoma.…”

Section: Treatmentmentioning

confidence: 99%

See 2 more Smart Citations

Non-Hodgkin lymphoma after pediatric kidney transplantation

Grenda

2021

Pediatr Nephrol

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Non-Hodgkin lymphoma (NHL) that develops after kidney transplantation belongs to post-transplant lymphoproliferative disorders (PTLD) occurring with an incidence of 2–3%. Most pediatric cases are related to primary infection with Epstein-Barr virus (EBV), able to transform and immortalize B cells and widely proliferate due to the lack of relevant control of cytotoxic T cells in patients receiving post-transplant immunosuppression. NHL may develop as a systemic disease or as a localized lesion. The clinical pattern is variable, from non-symptomatic to fulminating disease. Young age of transplant recipient, seronegative EBV status at transplantation, and EBV mismatch between donor and recipient (D+/R-) are regarded as risk factors. Immunosuppression impacts the development of both early and late NHLs. Specific surveillance protocols, including monitoring of EBV viral load, are used in patients at risk; however, detailed histopathology diagnosis and evaluation of malignancy staging is crucial for therapeutic decisions. Minimizing of immunosuppression is a primary management, followed by the use of rituximab in B-cell NHLs. Specific chemotherapeutic protocols, adjusted to lymphoma classification and staging, are used in advanced NHLs. Radiotherapy and/or surgical removal of malignant lesions is limited to the most severe cases. Outcome is variable, depending on risk factors and timing of diagnosis, however is positive in pediatric patients in terms of graft function and patient survival. Kidney re-transplantation is possible in survivors who lost the primary graft due to chronic rejection, however may be performed after at least 2–3 years of waiting time, careful verification of malignancy-free status, and gaining immunity against EBV.

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Non‐Hodgkin lymphoma in a kidney transplanted patient with methylmalonic acidemia: Metabolic susceptibility and the role of immunosuppression

Burlina,

Mignani

et al. 2024

JIMD Reports

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Methylmalonic acidemia cblB type (MMA cblB) is an autosomal recessive inborn error of amino acid metabolism that results in impaired synthesis of adenosylcobalamin, a cofactor of methylmalonyl‐CoA mutase. It presents with episodes of coma, vomiting, hypotonia, metabolic acidosis, and hyperammonemia. End‐stage kidney disease is a long‐term complication. Treatments include vitamin B12 supplementation, L‐carnitine, and a low‐protein diet. Liver, kidney, or combined liver‐kidney transplantations are promising options, but they are not without complications. We report a patient suffering from MMA cblB who developed end‐stage kidney disease at 18 years of age. Kidney transplantation allowed him to recover normal kidney function and good metabolic control. Unfortunately, after two decades, he developed non‐Hodgkin lymphoma and severe chemotherapy toxicity which led to his death. The risk of lymphoproliferative diseases is known to increase after solid organ transplantation. However, in MMA, factors including mitochondrial dysfunction and oncometabolites, may further increase the risk of malignancy and drug toxicity. Our report highlights the importance of considering the increased risk of cancer in long‐term follow‐up of MMA cblB patients, especially after solid organ transplantation. Moreover, when chemotherapy is needed, the increased risk of toxicity and metabolic decompensation should be considered and monitored.

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Joining Efforts for PTLD: Lessons Learned from Comparing the Approach and Treatment Strategies Across the Pediatric and Adult Age Spectra

Cited by 8 publications

References 38 publications

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

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

Non-Hodgkin lymphoma after pediatric kidney transplantation

Non‐Hodgkin lymphoma in a kidney transplanted patient with methylmalonic acidemia: Metabolic susceptibility and the role of immunosuppression

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