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Primary Mediastinal (Thymic) Large B-cell Lymphoma

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  • First Online:
Encyclopedia of Pathology

Part of the book series: Encyclopedia of Pathology ((EP))

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Synonyms

Mediastinal large B-cell lymphoma; Mediastinal lymphoma of clear cell type

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...

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References and Further Reading

  • 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.

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  • Chapuy, B. et al. (2018). Comprehensive Genomic analysis of primary mediastinal B-cell lymphoma (abstract). American society of hematology annual meeting. San Diego.

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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.

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Author information

Authors and Affiliations

  1. Department of Pathology, Brigham and Women’s Hospital, Boston, MA, USA

    Gabriel K. Griffin & Scott J. Rodig

Authors
  1. Gabriel K. Griffin
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  2. Scott J. Rodig
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Corresponding author

Correspondence to Gabriel K. Griffin .

Editor information

Editors and Affiliations

  1. NIJMEGEN, The Netherlands

    J.H.J.M. van Krieken

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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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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-28845-1

  • Online ISBN: 978-3-319-28845-1

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