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Mediastinal tumours
Published in Anju Sahdev, Sarah J. Vinnicombe, Husband & Reznek's Imaging in Oncology, 2020
Primary mediastinal B-cell lymphoma is a diffuse large B-cell lymphoma derived from the thymus. Common symptoms at presentation include chest pain, cough, dysphasia, superior vena cava syndrome, phrenic nerve palsy, and hoarseness (117). The involvement of extrathoracic structures and bone marrow is less common at presentation than for lymphoblastic lymphoma. However, on the recurrence of disease, involvement of the liver, kidneys, and brain can occur (121,122).
Haematology
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
The majority of childhood and adolescents NHL are high-grade and clinically aggressive. The range of histological subtypes is much narrower than seen in the adult population. There are essentially three subgroups: (i) precursor lymphoid neoplasms (T-cell lymphoblastic lymphoma [15–20%] and B-cell lymphoblastic lymphoma [3%]); (ii) mature B-cell neoplasms (Burkitt lymphoma [35–40%], diffuse large B-cell lymphoma [15–20%] and primary mediastinal B-cell lymphoma [1–2%]); and (iii) mature T-cell neoplasms (anaplastic large cell lymphoma, ALK positive [15–20%]).
Non-Hodgkin lymphoma
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2014
Piers Blombery, Adrian Bloor, David C. Linch
Classified as a separate disease entity in the current WHO classification, primary mediastinal (thymic) large B-cell lymphoma (PMBCL) usually presents in younger females with a large mediastinal mass.1 Pathologically, PMBCL is composed of large lymphocytes that express CD20, CD23 (a marker of thymic B-cells) and CD30 (weakly). The tumour often shows compartmentalizing sclerosis redolent of HL and indeed GEP data has shown PMBCL to be more similar to HL than to DLBCL.64 Other genetic abnormalities include 9p and 2p gains resulting in JAK2/REL amplification and JAK/STAT overactivity.
Rapid T-cell lymphoma progression associated with immune checkpoint inhibitors
Published in Expert Review of Hematology, 2023
Immunotherapy is the fourth pillar of cancer treatment, and immune checkpoint inhibitors (ICIs) are one of its branches. Considering the striking clinical features of melanoma or lung cancer, ICIs are currently prescribed to patients with various types of solid malignancies in clinical practice [1]. In cases of hematological malignancies, classical Hodgkin lymphoma (CHL) is the representative indication. Phase 2 trials of anti-programmed death-1 (PD1) antibody agents pembrolizumab or nivolumab for patients with relapsed/refractory CHL have exhibited overall response rates (ORRs) of 69% and 66%, respectively [2,3]. These results seem outstanding in that many enrolled patients experienced treatment failure after autologous hematopoietic stem-cell transplantation (ASCT). A phase 2 trial of nivolumab plus brentuximab vedotin for relapsed/refractory CHL patients in a post-ASCT consolidation setting has recently shown a progression-free survival rate of 94% at 18 months [4]. In addition, anti-PD1 antibodies are a promising treatment for relapsed/refractory primary mediastinal B-cell lymphoma (PMBCL). The rationale involves elevated programmed death-1 ligand (PD-L1) expression in PMBCL. Two trials of pembrolizumab showed ORRs of 48% and 45% [5].
Axicabtagene ciloleucel for the treatment of relapsed or refractory follicular lymphoma
Published in Expert Review of Anticancer Therapy, 2022
Building on this success, the NCI opened a single-arm phase 1 clinical trial utilizing the same anti-CD19 CAR T-cell product, FMC63-28Z (Clinical Trials.gov identifier: NCT00924326). Forty-three patients with relapsed B-cell malignancies were treated between 2009 and 2015 [53,54]. Twenty-eight patients had diffuse large B-cell lymphoma (DLBCL) or primary mediastinal B-cell lymphoma (PMBCL), seven had chronic lymphocytic leukemia (CLL), and eight had low-grade B-cell lymphoma including five with FL. Patients were heavily pretreated, with a median of four previous anti-lymphoma regimens among those with low-grade B-cell lymphoma. All five patients with FL had a response to treatment, three patients achieved a CR, and two achieved a PR. The long-term follow-up data at a median of 42 months showed a median event-free survival for all patients with low-grade B-cell lymphoma of 55 months; median OS was not reached. The percentage of CAR T-cell treatments resulting in a >3-year duration of response was 63% (95% CI, 25% to 92%) for low-grade lymphoma. Except for B-cell depletion and hypogammaglobulinemia, long-term adverse effects were rare. Median peak blood CAR-positive cell levels were higher among patients with a DOR of >3 years (98/mL; range, 9–1,217/mL) than among patients with a DOR of <3 years (18/mL; range, 0–308/mL, P = .0051).
Loncastuximab tesirine for treatment of relapsed or refractory diffuse large B cell lymphoma
Published in Expert Opinion on Biological Therapy, 2021
Krishna Goparaju, Paolo F. Caimi
LOTIS 2, multicenter, phase II trial included 145 patients with relapsed/ refractory DLBCL, with median age of 66 and slight male predominance accounting for 59% of patients. COO was unknown in 51% of patients, and the remaining were either GCB (33%) or ABC (16%) subtype. Of these patients, 15 patients had DHL/THL, 20 patients were double/triple expressors, 29 patients with transformed disease. Additionally, 11 patients (8%) with high grade B cell lymphoma (HGBCL) and 7 patients (5%) with primary mediastinal B cell lymphoma (PMBCL) were also included. Over two-thirds of patients had stage III–IV disease and had undergone at least 2 lines of systemic therapy, including prior HSCT in 24 patients and CAR-T 13 patients, prior to enrollment. 68% had primary relapse, 20% with primary refractory disease and 58% were refractory to their most recent systemic therapy. Patients with bulky disease (tumor ≥10 cm), active CNS lymphoma, Burkitt Lymphoma, recent HSCT within 60 days, prior loncastuximab exposure or history of hypersensitivity to CD-19 antibody were excluded.