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Advances in Chronic Lymphocytic Leukemia pp 1–24Cite as

B Cell Receptor and Antigens in CLL

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Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 792))

Abstract

Nowadays, chronic lymphocytic leukemia (CLL) is considered as a prototypic antigen-driven lymphoma, with antigenic stimuli from the microenvironment promoting tumor outgrowth. Antigen recognition is a function of both the clonotypic B cell receptor immunoglobulin (BcR IG) and various other immune sensors, e.g., the Toll-like receptors. The critical role of BcR IG-mediated signaling in CLL development and evolution is underscored by the following: the disease-biased IG gene repertoire; the subdivision of CLL based on the somatic hypermutation load of the BcR IG into two broad categories with vastly different prognosis and eventual outcome; the existence of subsets of cases with distinct, quasi-identical (stereotyped) BcR IGs; and the clinical efficacy of novel therapeutics inhibiting BcR signaling. Here, we trace the immunogenetic evidence for antigen selection in CLL and also consider the types of implicated antigens as well as the immune signaling pathways relevant for CLL ontogeny and clonal progression.

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Acknowledgments

We thank past and present members of our group for their commitment and enthusiasm. We also thank our fellow members in the IgCLL group, Drs. Belessi, Darzentas, Davi, Ghia, and Rosenquist, for our long-standing collaboration and stimulating scientific interaction.

This work was supported in part by the ENosAI project (code 09SYN-13-880) co-funded by the EU and the Hellenic General Secretariat for Research and Technology to KS; Cariplo Foundation (Milan, Italy) to KS.

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Authors and Affiliations

  1. B Cell Neoplasia Unit, Istituto Scientifico San Raffaele, Milan, Italy

    Andreas Agathangelidis

  2. Hematology Department and HCT Unit, G. Papanicolaou Hospital, Thessaloniki, 57010, Greece

    Stavroula Ntoufa & Kostas Stamatopoulos M.D.

  3. Institute of Applied Biosciences, Center for Research and Technology Hellas, Thessaloniki, Greece

    Stavroula Ntoufa & Kostas Stamatopoulos M.D.

Authors
  1. Andreas Agathangelidis
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  2. Stavroula Ntoufa
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  3. Kostas Stamatopoulos M.D.
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Correspondence to Kostas Stamatopoulos M.D. .

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Editors and Affiliations

  1. Department of Internal Medicine, University of Michigan, Ann Arbor, Michigan, USA

    Sami Malek

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Agathangelidis, A., Ntoufa, S., Stamatopoulos, K. (2013). B Cell Receptor and Antigens in CLL. In: Malek, S. (eds) Advances in Chronic Lymphocytic Leukemia. Advances in Experimental Medicine and Biology, vol 792. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-8051-8_1

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