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Non-Hodgkin Lymphoma
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
Piers Blombery, 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 tumor often shows compartmentalizing sclerosis redolent of HL and indeed GEP data have shown PMBCL to be more similar to HL than to DLBCL.47 Other recurrent genetic abnormalities in this entity include amplifications of 9p (involving JAK2 as well as CD274 [PD-L1] and PDCD1LG2 [PD-L2]) and oncogenic mutations involving the JAK/STAT and NF-kB pathways. 48
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.
Lung and Mediastinum
Published in Wojciech Gorczyca, Atlas of Differential Diagnosis in Neoplastic Hematopathology, 2014
Primary mediastinal (thymic) large B-cell lymphoma (PMBL) is subtype of DLBCL. PMBL accounts for approximately 5% of aggressive lymphomas and has a predilection for young women, bulky disease in the anterior-superior mediastinum (Figure 42.9), high LDH levels and frequent intrathoracic extension to adjacent organs such as pleura, pericardium, and lung [15–33]. Extrathoracic dissemination and bone marrow involvement are uncommon. Clinically, PMBL resembles classical HL. It is of thymic origin [34, 35]. PMBL has a relatively favorable clinical outcome, with a 5-year survival rate of 64% compared with 46% for other DLBCL patients [21].
Relapsed/refractory primary mediastinal large B-cell lymphoma: a structured review of epidemiology, treatment guidelines and real-world treatment practices
Published in Expert Review of Hematology, 2020
Jitender Takyar, Monika Raut, Rebekah Borse, Arun Balakumaran, Manu Sehgal
Primary mediastinal (thymic) large B-cell lymphoma (PMBCL) is an uncommon subtype of diffuse large B-cell lymphoma (DLBCL) that accounts for 3–10% of all DLBCLs and 2–4% of all non-Hodgkin lymphomas (NHL) [1–7]. Young adults in the third or fourth decade of life are most commonly affected, and a female predominance is typically observed [4,5,8]. With a probable origin in thymic B-cells [4,9,10], PMBCL typically presents as a bulky anterior mediastinal mass that often invades local thoracic structures, with many patients experiencing the resulting complications of pleural and pericardial effusions and superior vena cava obstruction [1,4–6,11].