Primary, Bilateral and Diffuse Renal Non-Hodgkin's Lymphoma in a Young Woman Suffering from Turner Syndrome

Primary renal lymphoma (PRL) is a rare form of non-Hodgkin's lymphoma (NHL) restricted to and primarily involving one or both kidneys, with no lymph node extension. It accounts for <1% of extranodal lymphomas, and descriptions in the literature are limited. Here, we describe an unprecedented case of bilateral PRL in a 44-year-old woman with Turner syndrome and discuss both diagnostic and therapeutic issues in the light of the available literature in the field. A personalized approach to this rare disease is necessary

PET/CT Imaging of Lymphoma Outside the Western World

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Impact of immunochemotherapy with R-bendamustine or R-CHOP for treatment naïve advanced-stage follicular lymphoma: A subset analysis of the FOLL12 trial by Fondazione Italiana Linfomi

: We conducted a post hoc analysis of the FOLL12 trial to determine the impact of different initial immunochemotherapy (ICT) regimens on patient outcomes. Patients were selected from the FOLL12 trial, which included adults with stage II-IV follicular lymphoma (FL) grade 1-3a and high tumor burden. Patients were randomized 1:1 to receive either standard ICT followed by rituximab maintenance (RM) or the same ICT followed by a response-adapted approach. ICT consisted of rituximab-bendamustine (RB) or rituximab, cyclophosphamide, doxorubicin, and prednisone (R-CHOP), per physician's decision. A total of 786 patients were included in this analysis, 341 of whom received RB and 445 R-CHOP. RB was more frequently prescribed to older subjects, females, patients without bulky disease, and those with grade 1-2 FL. After a median of 56 months of follow-up, R-CHOP and RB had similar progression-free survival (PFS) (Hazard Ratio for RB 1.11, 95% CI 0.87-1.42, p = 0.392). Standard RM was associated with improved PFS compared to response-adapted management both after R-CHOP and RB. Grade 3-4 hematologic adverse events were more frequent with R-CHOP during induction treatment and more frequent with RB during RM. Grade 3-4 infections were more frequent with RB. RB was also associated with a higher incidence of transformed FL. R-CHOP and RB showed similar activity and efficacy, but with different safety profiles and long-term events, suggesting that the treating physician should carefully select the most appropriate chemotherapy regimen for each patient based on patient's individual characteristics, choices, and risk profile

Response-Adapted Postinduction Strategy in Patients With Advanced-Stage Follicular Lymphoma: The FOLL12 Study

Purpose: We compared 2 years of rituximab maintenance (RM) with a response-adapted postinduction approach in patients with follicular lymphoma who responded to induction immunochemotherapy. Methods: We randomly assigned treatment-naïve, advanced-stage, high-tumor burden follicular lymphoma patients to receive standard RM or a response-adapted postinduction approach on the basis of metabolic response and molecular assessment of minimal residual disease (MRD). The experimental arm used three types of postinduction therapies: for complete metabolic response (CMR) and MRD-negative patients, observation; for CMR and MRD-positive (end of induction or follow-up) patients, four doses of rituximab (one per week, maximum three courses) until MRD-negative; and for non-CMR patients, one dose of ibritumomab tiuxetan followed by standard RM. The study was designed as noninferiority trial with progression-free survival (PFS) as the primary end point. Results: Overall, 807 patients were randomly assigned. After a median follow-up of 53 months (range 1-92 months), patients in the standard arm had a significantly better PFS than those in the experimental arm (3-year PFS 86% v 72%; P < .001). The better PFS of the standard vs experimental arm was confirmed in all the study subgroups except non-CMR patients (n = 65; P = .274). The 3-year overall survival was 98% (95% CI, 96 to 99) and 97% (95% CI, 95 to 99) in the reference and experimental arms, respectively (P = .238). Conclusion: A metabolic and molecular response-adapted therapy as assessed in the FOLL12 study was associated with significantly inferior PFS compared with 2-year RM. The better efficacy of standard RM was confirmed in the subgroup analysis and particularly for patients achieving both CMR and MRD-negative

T-CELL PROJECT: IL VALORE DEI REGISTRI LINFOMI A CELLULE T PERIFERICHE

Introduzione. I linfomi a cellule T periferiche (PTCL) sono un gruppo raro ed eterogeneo di neoplasie che hanno origine dalle cellule linfoidi post-timiche a diversi stadi di differenziazione. Differiscono tra di loro per caratteristiche morfologiche e fenotipiche, per la presentazione clinica, la risposta alle terapie e la prognosi. I PTCL rappresentano il 10-15% di tutti i disturbi linfoproliferativi nell'emisfero occidentale, con un'incidenza complessiva di 0,5-2 casi x100.000 persone/anno. La rarità ed eterogeneità dei PTCL ha reso estremamente difficile la loro conoscenza, e la ricerca è tuttora concentrata ad una maggiore comprensione del decorso clinico, della risposta al trattamento e della prognosi dei pazienti che ricevono una diagnosi di questo tipo. I PTCL colpiscono con maggiore frequenza i soggetti di sesso maschile, e l'età media alla diagnosi è di 62 anni. Secondo l’ultima classificazione dei linfomi pubblicata dalla nel 2016 (WHO2016) si possono distinguere più di 20 dversi sottotipi di PTCL, dei quali quelli più comuni sono: PTCL non altrimenti specificato (NOS; 25,9%), angioimmunoblastico (AITL; 18,5%), il linfoma nasale extranodale a cellule NK/T (ENKTCL; 10,4%), a cellule T dell'adulto leucemia/linfoma (ATLL; 9,6%), linfoma anaplastico a grandi cellule ALK positivo (ALCL, ALK+; 6,6%) e ALK negativo (ALCL, ALK-; 5,5%). Rispetto ai linfomi non-Hodgkin a cellule B, i PTCL sono associati a scarsa risposta alla terapia iniziale, ad alti tassi di recidiva e ad una prognosi sfavorevole, con un tasso di sopravvivenza a 5 anni inferiore al 40%. Metodi: Il T-cell Project 2.0 è uno studio prospettico prognostico internazionale di casi di PTCL di nuova diagnosi, che soddisfano i criteri di eleggibilità previsti da protocollo. E’ previsto l’arruolamento di 1.000 pazienti. I casi sono registrati su una piattaforma dedicata, e seguiti fino a 5 anni dalla diagnosi. L’endpoint primario dello studio è la sopravvivenza libera da progressione a due anni (2-year progression-free survival). Risultati. Da Maggio 2018 a Settembre 2021 sono stati registrati 738 casi da parte di 93 centri attivi in 14 Paesi, in 5 Continenti. Di questi, 694 casi sono risultati attualmente valutabili per l’endpoint primario. Da una analisi preliminare, i sottotipi più frequenti sono risultati essere il PTCL-NOS, il linfoma anaplastico a grandi cellule (ALCL) ALK-negativo e il linfoma angioimmunoblastico a cellule T (AITL), che rappresentano rispettivamente il 31%, il 19% e il 13% dei casi. Più in dettaglio, i PTCL-NOS rappresentano il sottotipo più frequente in tutte le arre geografiche, mentre la leucemia/linfoma a cellule T dell'adulto risulta la più frequente in Brasile, i sottotipi AITL e ALCL ALK-negativi in Australia/India e gli ALCL ALK-positivi in Nord America ed Europa. Inoltre, si è registrata una frequenza relativamente alta di casi di linfoma extranodale a cellule NK/T in Brasile, ma non negli altri Paesi dell'America Latina. I restanti sottotipi infine rappresentano meno del 5% dei casi in tutte le aree geografiche. Conclusioni. Data la rarità dei linfomi a cellule T periferiche, il T-cell Project 2.0, nato da una collaborazione internazionale, rappresenta una importante fonte di dati, che fotografano la prognosi e la curabilità di queste malattie nella Real Lyfe. Il TCP2 inoltre consentirà di meglio valutare la rilevanza clinica della Classificazione WHO2016, approfondire il ruolo della PET nella stadiazione e nella valutazione della risposta, e infine indagare sulle strategie di trattamento più adeguate per queste neoplasie.Background: Peripheral T-cell lymphomas (PTCLs) comprise a heterogeneous group of neoplasms that are derived from post-thymic lymphoid cells at different stages of differentiation with diverse morphological patterns, phenotypes, and clinical presentation. PTCLs account for 10–15% of all lymphoproliferative disorders in the Western hemisphere, with an overall incidence of 0.5–2/100,000 people/year, and have a striking epidemiological distribution, with higher incidence in Asia. The exceeding rarity (5–10% of all lymphoproliferative disorders) and the heterogeneity of PTCLs has made extremely difficult to investigate on them, and a satisfactory understanding of their clinical pictures, response to treatment and prognosis are still awaited. More commonly they appeared in male patients, and the median age at diagnosis is 62 years. In the last 2016 WHO classification there are more than 20 subtypes of PTLC, where the most common subtypes are PTCL not otherwise specified (NOS; 25.9%), angioimmunoblastic (AITL; 18.5%), NKTCL (10.4%), adult T-cell leukemia/lymphoma (ATLL) 9.6%, anaplastic large-cell lymphoma (ALCL) ALK positive (6.6%) and ALCL, ALK negative (5.5%). PTCLs are associated with high relapse rates and a poor prognosis compared to B-cell non-Hodgkin lymphomas with a 5-year-survival rate less than 40%. Objectives: In 2018, the International T-cell non-Hodgkin’s Lymphoma Study Group launched the T-cell Project 2.0 (TCP 2.0), which adapts to changes made in diagnosis, classification, staging and response evaluation, in order to verify whether a prospective collection of data would allow to achieve more accurate information on T-cell lymphomas and search for more disease oriented prognostic models. In particular, the aim of the TCP 2.0 relied on the opportunity of contributing to a real-time understanding of the evolving landscape of T-cell lymphoma biology and treatment, together with the application of recently available new technologies to further identify new therapeutic targets. Methods: Consecutive patients with newly diagnosed PTCLs according to the WHO2016 classification and satisfying inclusion criteria have been prospectively registered at a dedicated website via secure HTTP protocols, and followed for up to 5 years. Two-year Progression free survival was chosen as primary end point. Results. Since the beginning of the study on May 2018, 738 patients with newly diagnosed PTCL were registered by 93 active centers across 14 countries. Of these data on, 694 cases have been validated by the centralized trial office. Overall, PTCL-NOS, Anaplastic large cell lymphoma (ALCL) ALK-negative, and Angioimmunoblastic T-cell lymphoma (AITL), represent the most frequent subtypes, accounting on 31%, 19% and 13% of cases, respectively. Moreover, PTCL-NOS represents the most frequent subtype worldwide, whereas Adult T-cell leukemia/lymphoma was more frequent in Brazil, AITL and ALCL ALK-negative in Australia/India, and ALCL ALK-positive in North America and Europe. Of note, extranodal NK/T-cell lymphoma, nasal type, was relatively frequent in Brazil and quite rare in the other Latin America Countries. Finally, many sub-types represent less than 5% of cases in all geographic areas. Conclusions. The TCP2.0 continues to recruit very well, despite the difficulties linked to the COVD-19 pandemic, and represent a powerful source of data for better assessing the clinical relevance of the 2016 WHO Classification, the role of FDG-PET in staging and response assessment, the prognosis of different entities, the genomic landscape of different subtypes, and to investigate on the most adequate treatment strategies for these neoplasms in the real-world setting

Late Cardiological Sequelae and Long-Term Monitoring in Classical Hodgkin Lymphoma and Diffuse Large B-Cell Lymphoma Survivors: A Systematic Review by the Fondazione Italiana Linfomi

Cardiotoxicity represents the most frequent cause with higher morbidity and mortality among long-term sequelae affecting classical Hodgkin lymphoma (cHL) and diffuse large B-cell lymphoma (DLBCL) patients. The multidisciplinary team of Fondazione Italiana Linfomi (FIL) researchers, with the methodological guide of Istituto di Ricerche Farmacologiche “Mario Negri”, conducted a systematic review of the literature (PubMed, EMBASE, Cochrane database) according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, in order to analyze the following aspects of cHL and DLBCL survivorship: (i) incidence of cardiovascular disease (CVD); (ii) risk of long-term CVD with the use of less cardiotoxic therapies (reduced-field radiotherapy and liposomal doxorubicin); and (iii) preferable cardiovascular monitoring for left ventricular (LV) dysfunction, coronary heart disease (CHD) and valvular disease (VHD). After the screening of 659 abstracts and related 113 full-text papers, 23 publications were eligible for data extraction and included in the final sample. There was an increased risk for CVD in cHL survivors of 3.6 for myocardial infarction and 4.9 for congestive heart failure (CHF) in comparison to the general population; the risk increased over the years of follow-up. In addition, DLBCL patients presented a 29% increased risk for CHF. New radiotherapy techniques suggested reduced risk of late CVD, but only dosimetric studies were available. The optimal monitoring of LV function by 2D-STE echocardiography should be structured according to individual CV risk, mainly considering as risk factors a cumulative doxorubicine dose >250 mg per square meter (m2) and mediastinal radiotherapy >30 Gy, age at treatment 60 years, evaluating LV ejection fraction, global longitudinal strain, and global circumferential strain. The evaluation for asymptomatic CHD should be offered starting from the 10th year after mediastinal RT, considering ECG, stress echo, or coronary artery calcium (CAC) score. Given the suggested increased risks of cardiovascular outcomes in lymphoma survivors compared to the general population, tailored screening and prevention programs may be warranted to offset the future burden of disease

18F-FDG PET/CT Cannot Substitute Endoscopy in the Staging of Gastrointestinal Involvement in Mantle Cell Lymphoma. A Retrospective Multi-Center Cohort Analysis

The detection of gastrointestinal (GI) involvement in Mantle Cell Lymphoma is often underestimated and may have an impact on outcome and clinical management. We aimed to evaluate whether baseline 18F-FDG PET/CT presents comparable results to endoscopic biopsy in the diagnosis of GI localizations. In our retrospective cohort of 79 patients, sensitivity and specificity of 18F-FDG PET/CT were low for the stomach, with a fair concordance (k = 0.32), while higher concordance with pathologic results (k = 0.65) was detected in the colorectal tract. Thus, gastric biopsy remains helpful in the staging of MCL despite 18F-FDG PET/CT, while colonoscopy could be omitted in asymptomatic patients. The validation of our data in prospective cohorts is desirabl