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Diffuse Large B-Cell Lymphoma Associated with Chronic Inflammation and Fibrin-Associated Large B-Cell Lymphoma

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Precision Molecular Pathology of Aggressive B-Cell Lymphomas

Part of the book series: Molecular Pathology Library ((MPLB))

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Abstract

Diffuse large B-cell lymphoma associated with chronic inflammation (DLBCL-CI) is an Epstein-Barr virus (EBV)-associated lymphoma that arises in the setting of a prolonged inflammatory assault, usually within an enclosed, immune-sequestered site. The prototypical and most well-described example is pyothorax-associated lymphoma, which commonly occurs in the setting of treated tuberculosis and subsequent chronic pyothorax, but other clinical scenarios are described in the literature. Fibrin-associated large B-cell lymphoma (FA-LBCL) is non-mass forming and is commonly discovered in the setting of cardiac thrombi, myxomas, or prostheses, though other clinical scenarios are also described. Given overlapping features with DLBCL-CI, FA-LBCL was considered a subcategory of DLBCL-CI in the revised fourth edition of the World Health Organization (WHO 2016) classification. However, the differences in presentation, molecular features, and clinical outcomes warrant consideration of FA-LBCL as a separate entity, and this has been put forth in the new WHO fifth edition (WHO-HAEM5) classification and International Consensus Classification schemas.

DLBCL-CI is composed of large neoplastic lymphocytes of non-germinal center B-cell phenotype with expression of EBV antigens. Features overlap with other EBV-associated entities, but recognition of the distinct clinical presentation is a key determinant in diagnosis. TP53 mutations and MYC amplifications are common findings, but other aberrations exist, frequently with complex karyotypes. Unlike FA-LBCL, DLBCL-CI follows an aggressive clinical course which may vary depending on clinical and laboratory parameters.

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Authors and Affiliations

  1. Department of Pathology, University of New Mexico Health Sciences Center, Albuquerque, NM, USA

    Jonathon Gralewski & Daniel Babu

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  1. Jonathon Gralewski
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  2. Daniel Babu
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Correspondence to Daniel Babu .

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Editors and Affiliations

  1. Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH, USA

    Genevieve M. Crane

  2. Department of Hematopathology, The University of Texas, MD Anderson Cancer Center, Houston, TX, USA

    Sanam Loghavi

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Gralewski, J., Babu, D. (2023). Diffuse Large B-Cell Lymphoma Associated with Chronic Inflammation and Fibrin-Associated Large B-Cell Lymphoma. In: Crane, G.M., Loghavi, S. (eds) Precision Molecular Pathology of Aggressive B-Cell Lymphomas . Molecular Pathology Library. Springer, Cham. https://doi.org/10.1007/978-3-031-46842-1_21

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  • DOI: https://doi.org/10.1007/978-3-031-46842-1_21

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