Definition
Primary mediastinal large B-cell lymphoma (PMBL) is a large cell lymphoma of thymic medullary B-cell origin that exhibits clinicopathologic and molecular features that are more similar to classical Hodgkin lymphoma (CHL) than conventional diffuse large B-cell lymphoma (DLBCL). PMBL typically occurs in younger patients, shows a female predominance, and demonstrates characteristic genetic and immunophenotypic features related to activation of the NF-KB and JAK-STAT pathways. In addition, PMBL shows frequent structural alterations of chromosome 9p24 leading to upregulation of the immunoregulatory proteins PD-L1 and PD-L2. Due to these unique features, PMBL is considered as a distinct diagnostic category within the World Health Organization (WHO) Classification of Tumors of Hematopoietic and Lymphoid Tissues.
Clinical Features
Incidence
PMBL comprises approximately 2–4% of incident non-Hodgkin...
References and Further Reading
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Dorfman, D. M., et al. (2012). Utility of CD200 immunostaining in the diagnosis of primary mediastinal large B cell lymphoma: comparison with MAL, CD23, and other markers. Modern Pathology, 25(12), 1637–1643.
Dunleavy, K., et al. (2013). Dose-adjusted EPOCH-rituximab therapy in primary mediastinal B-cell lymphoma. The New England Journal of Medicine, 368(15), 1408–1416.
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Shi, M., et al. (2014). Expression of programmed cell death 1 ligand 2 (PD-L2) is a distinguishing feature of primary mediastinal (thymic) large B-cell lymphoma and associated with PDCD1LG2 copy gain. The American Journal of Surgical Pathology, 38(12), 1715–1723.
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Griffin, G.K., Rodig, S.J. (2019). Primary Mediastinal (Thymic) Large B-cell Lymphoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_3813-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_3813-1
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