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Supplemental material, sj-pdf-1-tmj-10.1177_03008916231185981 for Current practices of follow-up programs for childhood cancer survivors in Italy by Monica Terenziani, Alberto Eugenio Tozzi, Laura Diaco, Eleonora Biasin, Alessandro Cattoni, Ileana Croci, Donatella Fraschini, Giovanna Giorgiani, Riccardo Haupt, Monica Muraca, Marta Pillon, Giovanna Sironi, Maria Grazia Valsecchi and Angela Mastronuzzi in Tumori Journal

Supplemental material, sj-pdf-1-tmj-10.1177_03008916231185981 for Current practices of follow-up programs for childhood cancer survivors in Italy by Monica Terenziani, Alberto Eugenio Tozzi, Laura Diaco, Eleonora Biasin, Alessandro Cattoni, Ileana Croci, Donatella Fraschini, Giovanna Giorgiani, Riccardo Haupt, Monica Muraca, Marta Pillon, Giovanna Sironi, Maria Grazia Valsecchi and Angela Mastronuzzi in Tumori Journal

The European Intergroup for Childhood Non-Hodgkin Lymphoma (EICNHL) was established 25 years ago with the goal to facilitate clinical trials and research collaborations in the field both within Europe and worldwide. Since its inception, much progress has been made whereby major improvements in outcomes have been achieved. In this Review, we describe the different diagnostic entities of non-Hodgkin lymphoma in children and young adults describing key features of each entity and outlining clinical achievements made in the context of the EICNHL framework. Furthermore, we provide an overview of advances in biopathology with an emphasis on the role of biological studies and how they have shaped available treatments. Finally, for each entity, we describe future goals, upcoming clinical trials, and highlight areas of research that require our focus going forward.

Pediatric-type follicular (PTFL), marginal zone (MZL), and peripheral T-cell lymphoma (PTCL) account each for

The use of intensive chemotherapy regimens in children with lymphoblastic lymphoma (LBL) has significantly improved outcome, but the salvage rate for these patients is still poor. The aim of this study was to evaluate the prognostic value of minimal disseminated disease (MDD), studied by multiparametric flow cytometry (MFC), in pediatric patients with T- and B-lineage LBL.We examined bone marrow (BM) and peripheral blood (PB) samples from a series of 65 children affected by T- (52) and B-lineage (13) LBL using an MFC method; 10 of them were also analyzed for clonality of T-cell receptor gene rearrangements.MDD was detected in 49% (32/65) of BM samples, whereas only 21% (14/65) were positive at standard morphological evaluation. Findings from MFC analyses of paired BM and PB samples were highly concordant. We analyzed the prognostic significance of MDD results detected at diagnosis in morphologically negative patients, as almost all relapsed cases (10/11) did not have any morphological involvement of BM at diagnosis. Using an MDD cut-off level of 3% by FCM (75th percentile), 5-year event-free survival (EFS) was 60% (SE ± 22) for patients with MDD3% LBL cells versus 83% (SE ± 6) for the remaining patients (P = 0.04). No statistically significant difference in EFS was observed between LBL patients considering all the other clinical characteristics.Our data demonstrated that MDD studied at diagnosis by MFC could represent a useful prognostic tool in childhood LBL and further application for better stratification is warranted.

Despite poor survival, controversies remain in the treatment for refractory or relapsed pediatric non-Hodgkin lymphoma (r/r NHL). The current project aimed to collect international experience on the re-induction treatment of r/r NHL, hematopoietic stem cell transplantation (HSCT), risk factors associated with outcome, and to suggest treatment recommendations. Inclusion criteria were (i) refractory disease, disease progression or relapse of any NHL subtype except anaplastic large cell lymphoma, (ii) age < 18 years at initial diagnosis, (iii) diagnosis in/after January 2000. Data from 639 eligible patients were evaluable. The eight-year probability of overall survival was 34 ± 2% with highly significant differences according to NHL subtypes: 28 ± 3% for 254 Burkitt lymphoma/leukemia, 50 ± 6% for 98 diffuse large B-cell lymphomas, 57 ± 8% for 41 primary mediastinal large B-cell lymphomas, 27 ± 3% for 177 T-lymphoblastic lymphomas, 52 ± 10% for 34 precursor-B-cell lymphoblastic lymphomas and 30 ± 9% for 35 patients with rare NHL subtypes. Subtype-specific factors associated with survival and treatment recommendations are suggested. There were no survivors without HSCT, except in few very small subgroups. Conclusions: There is an urgent need to further improve survival in r/r NHL. The current study provides the largest real-world series, which underlines the role of HSCT and suggests treatment recommendations.

Background Many biological and inflammatory markers have been proposed as having a prognostic value at diagnosis of Hodgkin lymphoma (HL), but very few have been validated in paediatric patients. We explored the significance of these markers in a large population of 769 affected children. Patients and methods By using the database of patients enrolled in A.I.E.O.P. (Associazione Italiana di Emato-Oncologia Pediatrica) trial LH2004 for paediatric HL, we identified 769 consecutive patients treated with curative intent from 1st June 2004 to 1st April 2014 with ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine), or hybrid COPP/ABV (cyclophosphamide, vincristine, prednisone, procarbazine, doxorubicin, bleomycin and vinblastine) regimens. Results On multivariate analysis with categorical forms, the 5-year freedom from progression survival was significantly lower in patients with stage IV or elevated value of platelets, eosinophils and ferritin at diagnosis. Furthermore, stage IV and eosinophils seem to maintain their predictive value independently of interim (after IV cycles of chemotherapy) positron emission tomography. Conclusion Using the combination of four simple markers such as stage IV and elevated levels of platelets, ferritin and eosinophils, it is possible to classify the patients into subgroups with very different outcomes.

We analyzed the outcome of 537 adolescents (age 12-18 years) with idiopathic aplastic anemia included in the database of the Severe Aplastic Anemia Working Party of the European Group for Blood and Marrow Transplantation comparing: i) matched family donor hematopoietic stem cell transplantation performed as first-line treatment with ii) front-line immunosuppressive therapy not followed by subsequent transplant given for failure and with iii) hematopoietic stem cell transplantation performed after failed front-line immunosuppressive therapy. Overall survival was 86% in the matched family donor hematopoietic stem cell transplantation group, 90% in patients given front-line immunosuppressive alone (those who did not fail this treatment and who did not receive subsequent rescue with hematopoietic stem cell transplantation) and 78% in subjects who underwent hematopoietic stem cell transplantation post failed front-line immunosuppressive therapy (P=0.14). Event-free survival in the same groups was respectively 83%, 64% and 71% (P=0.04). Cumulative incidence of rejection was 8% in matched family donor hematopoietic stem cell transplantation and 9% in transplants post failed front-line immunosuppression (P=0.62). Cumulative incidence of acute graft-versus-host disease was 12% in matched family donor transplants and 18% in transplants post failed immunosuppression (P=0.18). Chronic graft-versus-host disease was higher in matched family donor hematopoietic stem cell transplantation (8%) than in transplants post failed immunosuppressive therapy (20%) (P=0.0009). Cumulative incidence of post-therapy malignancies was 0.7% in matched family donor transplantations, 7% in transplantations post failed immunosuppression and 21% after front-line immunosuppression (P=0.0017). In the whole cohort, under multivariate analysis, the diagnosis to treatment interval of two months or under positively affected overall survival whereas up-front immunosuppression alone (with no subsequent rescue transplants) negatively affected event-free survival. In transplanted patients an interval from diagnosis to treatment of 2 months or under, bone marrow as source of cells and first-line matched family donor transplants provided a significant advantage in overall and event-free survival. Aplastic anemia in adolescents has a very good outcome. If a matched family donor is available, hematopoietic stem cell transplantation using bone marrow cells is the first choice treatment. If such a donor is not available, immunosuppressive treatment may still be an acceptable second choice, also because, in case of failure, hematopoietic stem cell transplantation is a very good rescue option.