Encyclopedia of Pathology

Living Edition
| Editors: J.H.J.M. van Krieken

Primary Mediastinal (Thymic) Large B-cell Lymphoma

  • Gabriel K. GriffinEmail author
  • Scott J. Rodig
Living reference work entry
DOI: https://doi.org/10.1007/978-3-319-28845-1_3813-1



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...

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References and Further Reading

  1. Bledsoe, J. R., et al. (2016). The immunophenotypic spectrum of primary mediastinal large B-cell lymphoma reveals prognostic biomarkers associated with outcome. American Journal of Hematology, 91(10), E436–E441.CrossRefGoogle Scholar
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  3. Chapuy, B. et al. (2018). Comprehensive Genomic analysis of primary mediastinal B-cell lymphoma (abstract). American society of hematology annual meeting. San Diego.Google Scholar
  4. 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.CrossRefGoogle Scholar
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  12. Vigano, E., et al. (2018). Somatic IL4R mutations in primary mediastinal large B-cell lymphoma lead to constitutive JAK-STAT signaling activation. Blood, 131(18), 2036–2046.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of PathologyBrigham and Women’s HospitalBostonUSA