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Antigen receptor stereotypy in chronic lymphocytic leukemia
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Experimental and Clinical Oncology. Uppsala University, Science for Life Laboratory, SciLifeLab. Ctr Res & Technol Hellas, Inst Appl Biosci, 6th Km Charilaou Thermi Rd, Thessaloniki 57001, Greece.
Ctr Res & Technol Hellas, Inst Appl Biosci, 6th Km Charilaou Thermi Rd, Thessaloniki 57001, Greece.;Univ Vita Salute San Raffaele, Div Expt Oncol, Milan, Italy.;Univ Vita Salute San Raffaele, Dept Oncohematol, Milan, Italy.;IRCCS San Raffaele Sci Inst Milano, Milan, Italy..
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Experimental and Clinical Oncology. Uppsala University, Science for Life Laboratory, SciLifeLab.
Univ Vita Salute San Raffaele, Div Expt Oncol, Milan, Italy.;Univ Vita Salute San Raffaele, Dept Oncohematol, Milan, Italy.; IRCCS San Raffaele Sci Inst Milano, Milan, Italy..
2017 (English)In: Leukemia, ISSN 0887-6924, E-ISSN 1476-5551, Vol. 31, no 2, 282-291 p.Article, review/survey (Refereed) Published
Abstract [en]

The discovery of almost identical or 'stereotyped' B-cell receptor immunoglobulins (BcR IG) among unrelated patients with chronic lymphocytic leukemia (CLL) cemented the idea of antigen selection in disease ontogeny and evolution. The systematic analysis of the stereotypy phenomenon in CLL revealed that around one-third of CLL patients may be grouped into subsets based on shared sequence motifs within the variable heavy complementarity determining region 3. Stereotyped subsets display a strikingly similar biology of the leukemic clones, referring to many different levels, from the immunogenetic and genetic and extending to the epigenetic and functional levels. Even more importantly, the homogeneity of stereotyped subsets has clinical consequences as patients assigned to the same stereotyped subset generally exhibit an overall similar disease course and outcome. In other words, stereotypy-based patient classification of CLL has already provided a more compartmentalized view of this otherwise heterogeneous disease and can assist in refining prognostication models. While this is relevant only for the one-third of cases expressing stereotyped BcR IG; in principle, however, the findings from further analysis of the stereotyped subsets may also contribute towards improved understanding of the remaining non-stereotyped fraction of CLL patients.

Place, publisher, year, edition, pages
NATURE PUBLISHING GROUP , 2017. Vol. 31, no 2, 282-291 p.
National Category
Hematology Cancer and Oncology
Identifiers
URN: urn:nbn:se:uu:diva-320092DOI: 10.1038/leu.2016.322ISI: 000394058800003PubMedID: 27811850OAI: oai:DiVA.org:uu-320092DiVA: diva2:1091722
Funder
Swedish Cancer SocietySwedish Research Council
Available from: 2017-04-27 Created: 2017-04-27 Last updated: 2017-04-27Bibliographically approved

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