4.7 Review

Primary mediastinal large B-cell lymphoma

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Summary: In the trial of using BV-R-CHP combination therapy for CD30-positive B-cell lymphomas, toxicity was limited with no treatment-related deaths, high overall response rate and complete response rate, high utilization of consolidative radiation, and ideal 2-year survival rates.

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Summary: The mutational landscape of gray zone lymphoma (GZL) differs between cases with and without thymic niche involvement, showing distinct patterns related to apoptosis defects and BCL2/BCL6 rearrangements, respectively. Additionally, poly-EBV-L cases exhibit a unique mutational profile with STAT3 mutations and lower coding mutation load compared to EBV- GZL. These findings suggest common cell of origin and disease evolution among GZL with thymic presentation and related anterior mediastinal lymphomas.
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Summary: The dose-adjusted etoposide, doxorubicin, and cyclophosphamide with vincristine and prednisone plus rituximab (DA-EPOCH-R) regimen did not show improved event-free survival compared to historical controls in children and adolescents with primary mediastinal large B-cell lymphoma (PMLBL) in the first prospective multisite international study, indicating the need for further research to determine the optimal therapy for this lymphoma in young patients.

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Summary: End-of-treatment PET/CT is essential in guiding the decision to omit radiotherapy for patients with primary mediastinal large B-cell lymphoma. Evaluating patients based on the Deauville 5-point scale criteria can predict their freedom from progression. The study suggests that a significant number of PET/CT-negative patients can safely avoid radiotherapy, and emphasizes the importance of not initiating salvage chemotherapy based solely on positive PET/CT results.

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Summary: Our investigation revealed that non-mediastinal PMBL-like lymphomas are a unique subgroup within DLBCL, showing distinct clinical and genetic features compared to conventional PMBL and DLBCL. These findings have potential implications for the subtyping of aggressive B-cell lymphomas and the development of targeted therapies.
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TNFAIP3 (A20) is a tumor suppressor gene in Hodgkin lymphoma and primary mediastinal B cell lymphoma

Roland Schmitz et al.

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