Burkitt lymphoma after solid-organ transplant: Treatment and outcomes in the paediatric PTLD collaborative.

Burkitt lymphoma arising in paediatric post-solid-organ transplantation-Burkitt lymphoma (PSOT-BL) is a clinically aggressive malignancy and a rare form of post-transplant lymphoproliferative disorder (PTLD). We evaluated 35 patients diagnosed with PSOT-BL at 14 paediatric medical centres in the United States. Median age at organ transplantation was 2.0 years (range: 0.1-14) and age at PSOT-BL diagnosis was 8.0 years (range: 1-17). All but one patient had late onset of PSOT-BL (≥2 years post-transplant), with a median interval from transplant to PSOT-BL diagnosis of 4.0 years (range: 0.4-12). Heart (n = 18 [51.4%]) and liver (n = 13 [37.1%]) were the most frequently transplanted organs. No patients had loss of graft or treatment-related mortality. A variety of treatment regimens were used, led by intensive Burkitt lymphoma-specific French-American-British/Lymphomes Malins B (FAB/LMB), n = 13 (37.1%), and a low-intensity regimen consisting of cyclophosphamide, prednisone and rituximab (CPR) n = 12 (34.3%). Median follow-up was 6.7 years (range: 0.5-17). Three-year event-free and overall survival were 66.2% and 88.0%, respectively. Outcomes of PSOT-BL patients receiving BL-specific intensive regimens are comparable to reported BL outcomes in immunocompetent children. Multi-institutional collaboration is feasible and provides the basis of prospective data collection to determine the optimal treatment regimen for PSOT-BL.

Multicenter study of pediatric Epstein-Barr virus-negative monomorphic post solid organ transplant lymphoproliferative disorders.

BACKGROUND: Pediatric Epstein-Barr virus-negative monomorphic post solid organ transplant lymphoproliferative disorder [EBV(-)M-PTLD] comprises approximately 10% of M-PTLD. No large multi-institutional pediatric-specific reports on treatment and outcome are available. METHODS: A multi-institutional retrospective review of solid organ recipients diagnosed with EBV(-)M-PTLD aged ≤21 years between 2001 and 2020 in 12 centers in the United States and United Kingdom was performed, including demographics, staging, treatment, and outcomes data. RESULTS: Thirty-six patients were identified with EBV(-)M-PTLD. Twenty-three (63.9%) were male. Median age (range) at transplantation, diagnosis of EBV(-)M-PTLD, and interval from transplant to PTLD were 2.2 years (0.1-17), 14 years (3.0-20), and 8.5 years (0.6-18.3), respectively. Kidney (n = 17 [47.2%]) and heart (n = 13 [36.1%]) were the most commonly transplanted organs. Most were Murphy stage III (n = 25 [69.4%]). Lactate dehydrogenase was elevated in 22/34 (64.7%) and ≥2 times upper limit of normal in 11/34 (32.4%). Pathological diagnoses included diffuse large B-cell lymphoma (n = 31 [86.1%]) and B-non-Hodgkin lymphoma (B-NHL) not otherwise specified (NOS) (n = 5 [13.9%]). Of nine different regimens used, the most common were: pediatric mature B-NHL-specific regimen (n = 13 [36.1%]) and low-dose cyclophosphamide, prednisone, and rituximab (n = 9 [25%]). Median follow-up from diagnosis was 3.0 years (0.3-11.0 years). Three-year event-free survival (EFS) and overall survival (OS) were 64.8% and 79.9%, respectively. Of the seven deaths, six were from progressive disease. CONCLUSIONS: EFS and OS were comparable to pediatric EBV(+) PTLD, but inferior to mature B-NHL in immunocompetent pediatric patients. The wide range of therapeutic regimens used directs our work toward developing an active multi-institutional registry to design prospective studies. PLAIN LANGUAGE SUMMARY: Pediatric Epstein-Barr virus-negative monomorphic post solid organ transplant lymphoproliferative disorders (EBV(-)M-PTLD) have comparable outcomes to EBV(+) PTLD, but are inferior to diffuse large B-cell lymphoma in immunocompetent pediatric patients. The variety of treatment regimens used highlights the need to develop a pediatric PTLD registry to prospectively evaluate outcomes. The impact of treatment regimen on relapse risk could not be assessed because of small numbers. In the intensive pediatric B-non-Hodgkin lymphoma chemoimmunotherapy group, 11 of 13 patients remain alive in complete remission after 0.6 to 11 years.

Implementing a pediatric oncology nursing multisite trial.

PURPOSE/BACKGROUND: The Parent Educational Discharge Support Strategies (PEDSS) nursing study includes 16 magnet pediatric oncology institutions across the United States and one in Saudi Arabia, evaluating a nurse-led parent educational discharge support strategy for families experiencing a child newly diagnosed with cancer. METHODS: During the first 3 months of the study, a research implementation survey was administered electronically to each site principal investigator to evaluate facilitators and barriers in the research process for this multisite nurse-led pediatric oncology study. RESULTS: Facilitators included nursing leadership support and commitment from the nursing staff. Common barriers reported were the Institutional Review Board process, the consent process, the timing of the intervention, data collection, as well as nursing time for the study. Results from the survey suggest nurse-led research teams were motivated and felt the intervention was easy to deliver. PRACTICE IMPLICATIONS: Nursing practice is enhanced when nurses participate in research and generate evidence regarding best practices within pediatric oncology nursing care. CONCLUSION: Nursing research endeavors focusing on collaborative approaches for implementation can lead to successful nursing studies with careful planning, training and administrative support.

Factors Affecting Adolescents' Willingness to Communicate Symptoms During Cancer Treatment: A Systematic Review from the Children's Oncology Group.

The purpose of this systematic review is to (1) synthesize evidence facilitators and barriers affecting adolescent willingness to communicate symptoms to health care providers (HCPs) and (2) create practice recommendations. The PICOT (Population, Intervention, Comparison, Outcome, Time) question guiding the review was, among adolescents with cancer, what factors affect their willingness to communicate symptoms to HCPs? Three databases, PubMed, CINAHL, and PsychINFO, were searched using keywords from the PICOT question. Inclusion criteria included original research studies with samples of at least 51% adolescents aged 10-18 years who were receiving or had received cancer treatment. Studies were included that evaluated outcomes related to willingness to communicate in general as well as communication of specific treatment symptoms. Exclusion criteria included systematic reviews and publications emphasizing cancer screening, cancer prevention, survivorship, or decision making. A total of 11 studies were included in the systematic review. Two of the studies focused on symptom communication; the remaining nine studies focused on general communication or the overlap between general and symptom communication. Barriers to symptom and/or general communication included negative expectations, health-related uncertainty, ambiguousness with assessments, unfamiliarity, restriction of information, perceived negative provider characteristics/behaviors, adolescent circumstance, worry about others' perceptions, and provider approach. Facilitators to symptom and/or general communication included open communication, perceived favorable provider characteristics/behaviors, seasoned adolescent, and patient-provider rapport. Five practice recommendations were developed from the evidence that supports general and symptom communication between adolescents who are receiving or have completed cancer treatment and their HCPs.