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Pathology and Molecular Pathogenesis of Burkitt Lymphoma and Lymphoblastic Lymphoma

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Aggressive Lymphomas

Part of the book series: Hematologic Malignancies ((HEMATOLOGIC))

Abstract

Burkitt lymphoma and lymphoblastic lymphoma are highly aggressive lymphomas mostly occurring in children, adolescents, and young adults. They account for approximately 4–5% of all non-Hodgkin lymphomas in Western countries. These B-cell neoplasms were frequently grouped together in the past, due to their fast-growing clinical behavior, related to a short doubling time, and their frequent presentation as acute leukemia. Burkitt leukemia was formerly classified as a lymphoblastic leukemia with L3 morphology according to the FAB classification. However, the biological characterization, especially the immunophenotype of these lymphomas, showed that Burkitt leukemia/lymphoma and lymphoblastic leukemia/lymphoma correspond to radically different stages of lymphoid maturation, with a different cell of origin, justifying their classification as separate entities in the WHO classification: lymphoblastic lymphoma is a B- or T-cell precursor lymphoid neoplasm, while Burkitt lymphoma is a mature B-cell neoplasm. As with other subtypes of lymphomas, emerging data from gene expression profiling, next-generation sequencing, and related techniques help to define these entities more precisely and to better understand their pathogenesis in order to identify potential new rational therapeutic targets.

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Authors and Affiliations

  1. Belgian Cancer Registry and GenHAP, Brussels, Belgium

    Hélène A. Poirel

  2. Section of Pathology, Department of Medical Biotechnology, University of Siena, Siena, Italy

    Maria Raffaella Ambrosio & Lorenzo Leoncini

  3. Department of Experimental, Diagnostic, and Specialty Medicine, University of Bologna, Bologna, Italy

    Pier Paolo Piccaluga

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  1. Hélène A. Poirel
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  2. Maria Raffaella Ambrosio
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  3. Pier Paolo Piccaluga
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  4. Lorenzo Leoncini
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Editors and Affiliations

  1. Department of Translational Oncology, University-Hospital Münster, Münster, Germany

    Georg Lenz

  2. Department of Hematology, University Claude Bernard Lyon 1, Pierre-Benite cedex, France

    Gilles Salles

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Poirel, H.A., Ambrosio, M.R., Piccaluga, P.P., Leoncini, L. (2019). Pathology and Molecular Pathogenesis of Burkitt Lymphoma and Lymphoblastic Lymphoma. In: Lenz, G., Salles, G. (eds) Aggressive Lymphomas. Hematologic Malignancies. Springer, Cham. https://doi.org/10.1007/978-3-030-00362-3_3

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  • DOI: https://doi.org/10.1007/978-3-030-00362-3_3

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